Presented by Jonathan Epstein and prepared by Sintawat Wangsiricharoen
A 68 year old female underwent a TUR of a large bladder mass
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A 68 year old female underwent a TUR of a large bladder mass.
Choose the correct diagnosis.
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Answer: B. Lymphoepithelioma-like urothelial carcinoma
Histological Description: There is a brisk benign lymphocytic infiltrate with scattered large nuclei with multiple nucleoli, abundant eosinophilic to amphophilic cytoplasm. These nuclei have scattered mitotic figures, are variably hyperchromatic, and some have irregular shapes.
Discussion: The major differential diagnosis in this case is between large cell lymphoma and lymphoepithelioma-like urothelial carcinoma (LELC). In this case CK7 and GATA3 were positive in the large atypical cells, which were negative for CD45 and CD20, diagnostic of LELC. In order to diagnosis LELC, it is not sufficient to just have a lymphocytic infiltrate associated with invasive urothelial carcinoma. Rather, in LELC sheets, cords, trabeculae of cells are almost effaced by the inflammatory infiltrate closely mimicking lymphoma. In contrast to lymphoepithelioma in the head and neck, LELCs are EBV negative. If the tumor is pure lymphoepithelioma-like carcinoma it may respond to chemotherapy such that cystectomy can be avoided. When LELC is mixed with conventional urothelial carcinoma, their outcome is similar to that for conventional urothelial carcinoma and depends on the stage of the associated carcinoma. Consequently, it is critical to recognize pure LELC as radical cystectomy can be avoided in the presence of muscularis propria invasion.
Incorrect
Answer: B. Lymphoepithelioma-like urothelial carcinoma
Histological Description: There is a brisk benign lymphocytic infiltrate with scattered large nuclei with multiple nucleoli, abundant eosinophilic to amphophilic cytoplasm. These nuclei have scattered mitotic figures, are variably hyperchromatic, and some have irregular shapes.
Discussion: The major differential diagnosis in this case is between large cell lymphoma and lymphoepithelioma-like urothelial carcinoma (LELC). In this case CK7 and GATA3 were positive in the large atypical cells, which were negative for CD45 and CD20, diagnostic of LELC. In order to diagnosis LELC, it is not sufficient to just have a lymphocytic infiltrate associated with invasive urothelial carcinoma. Rather, in LELC sheets, cords, trabeculae of cells are almost effaced by the inflammatory infiltrate closely mimicking lymphoma. In contrast to lymphoepithelioma in the head and neck, LELCs are EBV negative. If the tumor is pure lymphoepithelioma-like carcinoma it may respond to chemotherapy such that cystectomy can be avoided. When LELC is mixed with conventional urothelial carcinoma, their outcome is similar to that for conventional urothelial carcinoma and depends on the stage of the associated carcinoma. Consequently, it is critical to recognize pure LELC as radical cystectomy can be avoided in the presence of muscularis propria invasion.
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen
A 48 year old male underwent an orchiectomy for a testicular mass
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A 48 year old male underwent an orchiectomy for a testicular mass.
Choose the correct diagnosis.
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Answer: C. Malakoplakia
Histological Description: Focally residual necrotic seminiferous tubules are visible with associated marked acute inflammation. Adjacent to the extensive necrosis, are numerous cells with abundant eosinophilic cytoplasm with round nuclei with small but visible nucleoli. Within many of these cells are light amphophilic inclusions, some of which have the appearance of a target with a central dark round bulls-eye surrounded but a white circle. Admixed are numerous plasma cells, lymphocytes, neutrophils, and fibrosis.
Discussion: The key to the diagnosis in the case rests on recognizing that the overall process is inflammatory as opposed to neoplastic. Whereas seminomas typically have a lymphocytic infiltrate and can have a prominent associated granulomatous component, they lack admixed neutrophils. IgG 4 related disease is not well recognized in the testis and also would lack prominent neutrophils and necrosis. The tip to recognizing malacoplakia is noting that there are scattered bluish inclusions in many of the histiocytes and then searching for more classic small basophilic extracytoplasmic or intracytoplasmic calculopherules resembling bulls-eyes on a target (Michaelis-Gutmann bodies). Although typically these stains are usually not necessary, Michaelis-Gutmann bodies are positive for calcium stains (von Kossa) and iron stains (Prussian blue). Cells are positive for histiocyte markers (i.e. CD68) and negative for keratins. Treatment is primarily based on controlling the urinary tract infections, which stabilizes the disease. Adding bethanechol, a cholinergic agent thought to increase the intracellular cyclic guanosine monophosphate levels considered to be the defect-causing macrophage dysfunction, may also be useful. Surgery may be necessary, as in this case, if malacoplakia makes a mass mimicking a neoplasm or if it results in local morbidity that cannot be controlled with antibiotics.
Incorrect
Answer: C. Malakoplakia
Histological Description: Focally residual necrotic seminiferous tubules are visible with associated marked acute inflammation. Adjacent to the extensive necrosis, are numerous cells with abundant eosinophilic cytoplasm with round nuclei with small but visible nucleoli. Within many of these cells are light amphophilic inclusions, some of which have the appearance of a target with a central dark round bulls-eye surrounded but a white circle. Admixed are numerous plasma cells, lymphocytes, neutrophils, and fibrosis.
Discussion: The key to the diagnosis in the case rests on recognizing that the overall process is inflammatory as opposed to neoplastic. Whereas seminomas typically have a lymphocytic infiltrate and can have a prominent associated granulomatous component, they lack admixed neutrophils. IgG 4 related disease is not well recognized in the testis and also would lack prominent neutrophils and necrosis. The tip to recognizing malacoplakia is noting that there are scattered bluish inclusions in many of the histiocytes and then searching for more classic small basophilic extracytoplasmic or intracytoplasmic calculopherules resembling bulls-eyes on a target (Michaelis-Gutmann bodies). Although typically these stains are usually not necessary, Michaelis-Gutmann bodies are positive for calcium stains (von Kossa) and iron stains (Prussian blue). Cells are positive for histiocyte markers (i.e. CD68) and negative for keratins. Treatment is primarily based on controlling the urinary tract infections, which stabilizes the disease. Adding bethanechol, a cholinergic agent thought to increase the intracellular cyclic guanosine monophosphate levels considered to be the defect-causing macrophage dysfunction, may also be useful. Surgery may be necessary, as in this case, if malacoplakia makes a mass mimicking a neoplasm or if it results in local morbidity that cannot be controlled with antibiotics.
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen
A 55 year old man underwent a radical nephrectomy for a 5 cm renal mass
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A 55 year old man underwent a radical nephrectomy for a 5 cm renal mass.
Choose the correct diagnosis.
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Answer: B. Oncocytoma
Histological Description: Focally the tumor is composed of nests with large, round-to-polygonal cells with abundant eosinophilic cytoplasm in fibromyxoid stroma. In this area well-preserved nuclei that have fine chromatin are uniformly round with variably sized nucleoli. The majority of the tumor is composed of cords and irregular nests of pleomorphic cells in a fibromyxoid stroma. The pleomorphic cells have degenerative nuclear atypia with multinucleation, pseudoinclusions, and smudgy chromatin. Mitotic figures and necrosis are absent.
Discussion: Oncocytomas are typically composed of cells are arranged in nests, acini, tubules or microcysts set in a fibromyxoid stroma. In some cases lacking fibromyxoid stroma, there are still back-to-back nests; a diffuse growth pattern rules out oncocytoma. In the typical case of oncocytoma, well-preserved nuclei that with fine chromatin are uniformly round with variably sized nucleoli. Pyknotic dark nuclei with crenate irregular edges should be discounted. Although oncocytomas are benign, there are certain histological features that can mimic renal cell carcinoma. Extension into perirenal adipose tissue can be seen in 20% of cases and not an indication of malignancy. Invasion of renal sinus and renal vein branches can rarely be seen in typical oncocytomas and still consistent with a benign clinical course. Cells with degenerative atypia, as seen in this case, can also mimic malignancy. Typically, the cells with degenerative atypia occur in clusters surrounded by more classic appearing oncocytoma. Unusually, as in this case, the degenerative atypia is more diffuse and even more closely can be confused with renal cell carcinoma. Despite the prominent pleomorphism, there are no mitotic figures or necrosis, which would be expected if this was a pleomorphic grade 4 renal cell carcinoma. If one needed reassurance, a ki67 would be negative in the pleomorphic nuclei indicating a degenerative phenomenon.
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Answer: B. Oncocytoma
Histological Description: Focally the tumor is composed of nests with large, round-to-polygonal cells with abundant eosinophilic cytoplasm in fibromyxoid stroma. In this area well-preserved nuclei that have fine chromatin are uniformly round with variably sized nucleoli. The majority of the tumor is composed of cords and irregular nests of pleomorphic cells in a fibromyxoid stroma. The pleomorphic cells have degenerative nuclear atypia with multinucleation, pseudoinclusions, and smudgy chromatin. Mitotic figures and necrosis are absent.
Discussion: Oncocytomas are typically composed of cells are arranged in nests, acini, tubules or microcysts set in a fibromyxoid stroma. In some cases lacking fibromyxoid stroma, there are still back-to-back nests; a diffuse growth pattern rules out oncocytoma. In the typical case of oncocytoma, well-preserved nuclei that with fine chromatin are uniformly round with variably sized nucleoli. Pyknotic dark nuclei with crenate irregular edges should be discounted. Although oncocytomas are benign, there are certain histological features that can mimic renal cell carcinoma. Extension into perirenal adipose tissue can be seen in 20% of cases and not an indication of malignancy. Invasion of renal sinus and renal vein branches can rarely be seen in typical oncocytomas and still consistent with a benign clinical course. Cells with degenerative atypia, as seen in this case, can also mimic malignancy. Typically, the cells with degenerative atypia occur in clusters surrounded by more classic appearing oncocytoma. Unusually, as in this case, the degenerative atypia is more diffuse and even more closely can be confused with renal cell carcinoma. Despite the prominent pleomorphism, there are no mitotic figures or necrosis, which would be expected if this was a pleomorphic grade 4 renal cell carcinoma. If one needed reassurance, a ki67 would be negative in the pleomorphic nuclei indicating a degenerative phenomenon.
Presented by Dr. Ezra Baraban and prepared by Dr. Sintawat Wangsiricharoen
A male infant with a paratesticular mass
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A male infant with a paratesticular mass
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Answer: B. Melanotic neuroectodermal tumor of infancy
Sections show a biphasic neoplasm involving the epididymis composed of nests of small round blue primitive cells with interspersed cords and tubules of epithelioid cells containing melanin pigment. Adenomatoid tumor is the most common paratesticular tumor in adults, but it is mesothelial in nature and lacks small round blue cell morphology and lacks pigment. Metastatic neuroblastoma is a consideration but the absence of a primary lesion elsewhere as well as negativity for PHOX2b argue against this. Embryonal rhabdomyosarcoma is the most common malignant paratesticular tumor in pediatric patients, but desmin and myogenin reactivity would be expected. This tumor shows classic morphology of melanotic neuroectodermal tumor of infancy, which most commonly occurs in the head and neck, but has been reported in the paratesticular region. The primitive blue cell component shows synaptophysin labeling and the epithelioid, pigmented component expresses cytokeratin and HMB-45. Metastatic melanoma is vanishingly rare in this age group, particularly without a known primary, and would be expected to show S100 positivity while typically lacking keratin expression.
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Answer: B. Melanotic neuroectodermal tumor of infancy
Sections show a biphasic neoplasm involving the epididymis composed of nests of small round blue primitive cells with interspersed cords and tubules of epithelioid cells containing melanin pigment. Adenomatoid tumor is the most common paratesticular tumor in adults, but it is mesothelial in nature and lacks small round blue cell morphology and lacks pigment. Metastatic neuroblastoma is a consideration but the absence of a primary lesion elsewhere as well as negativity for PHOX2b argue against this. Embryonal rhabdomyosarcoma is the most common malignant paratesticular tumor in pediatric patients, but desmin and myogenin reactivity would be expected. This tumor shows classic morphology of melanotic neuroectodermal tumor of infancy, which most commonly occurs in the head and neck, but has been reported in the paratesticular region. The primitive blue cell component shows synaptophysin labeling and the epithelioid, pigmented component expresses cytokeratin and HMB-45. Metastatic melanoma is vanishingly rare in this age group, particularly without a known primary, and would be expected to show S100 positivity while typically lacking keratin expression.
Presented by Dr. Ezra Baraban and prepared by Dr. Sintawat Wangsiricharoen
An adult male with a kidney mass
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An adult male with a kidney mass
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Answer: C. Dedifferentiated liposarcoma
Sections show a low-grade spindle cell tumor with admixed collagen. Scattered mitotic activity including atypical forms and focal necrosis is noted. The differential diagnosis is broad, and includes sarcomatoid carcinoma (urothelial or renal cell carcinoma) and various sarcomas. Lack of primary carcinoma elsewhere and the absence of an epithelial component argue against sarcomatoid carcinoma. Initially this tumor was interpreted as a renal mass and favored to represent renal cell carcinoma by imaging but dedifferentiated liposarcoma can abut the kidney and mislead radiologists. In this case, no stains were required because other sections showed well-developed areas of well-differentiated liposarcoma. Atypical mitotic figures would be unusual for neoplasms with simple karyotypes such as fibromatosis and low-grade fibromyxoid sarcoma, with negativity for MUC4 essentially excluding the latter. Keratin and PAX8 expression would support sarcomatoid renal cell carcinoma, but identification of a well-differentiated liposarcoma component is diagnostic of dedifferentiated liposarcoma in this case. The morphologic spectrum of dedifferentiated liposarcoma is immense and should be considered for any low or high-grade mesenchymal neoplasm, particularly in visceral sites. Identification of well-differentiated liposarcoma or MDM2 amplification or overexpression are confirmatory.
Incorrect
Answer: C. Dedifferentiated liposarcoma
Sections show a low-grade spindle cell tumor with admixed collagen. Scattered mitotic activity including atypical forms and focal necrosis is noted. The differential diagnosis is broad, and includes sarcomatoid carcinoma (urothelial or renal cell carcinoma) and various sarcomas. Lack of primary carcinoma elsewhere and the absence of an epithelial component argue against sarcomatoid carcinoma. Initially this tumor was interpreted as a renal mass and favored to represent renal cell carcinoma by imaging but dedifferentiated liposarcoma can abut the kidney and mislead radiologists. In this case, no stains were required because other sections showed well-developed areas of well-differentiated liposarcoma. Atypical mitotic figures would be unusual for neoplasms with simple karyotypes such as fibromatosis and low-grade fibromyxoid sarcoma, with negativity for MUC4 essentially excluding the latter. Keratin and PAX8 expression would support sarcomatoid renal cell carcinoma, but identification of a well-differentiated liposarcoma component is diagnostic of dedifferentiated liposarcoma in this case. The morphologic spectrum of dedifferentiated liposarcoma is immense and should be considered for any low or high-grade mesenchymal neoplasm, particularly in visceral sites. Identification of well-differentiated liposarcoma or MDM2 amplification or overexpression are confirmatory.
Presented by Dr. Ezra Baraban and prepared by Dr. Sintawat Wangsiricharoen
A young adult male with an abdominal mass
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A young adult male with an abdominal mass
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Answer: E. Paraganglioma
Sections show an epithelioid neoplasm arranged in discrete small nests abutting the aorta. The cells are eosinophilic with speckled chromatin and there are numerous large multinucleated hyperchromatic cells. The morphology is the typical “zellballen” appearance of a paraganglioma, the term used for tumors arising from extra-adrenal autonomic tissue. This diagnosis is critical not to overlook because tumors often produce catecholamines leading to dangerous episodes of hypertension which may be exacerbated by surgical manipulation of the tumor. This entity has a very high correlation with germline genetic syndromes and genetic counseling is recommended for all patients. Screening for a subset of syndromes can be achieved with immunohistochemistry for SDH-B, which should be lost if the tumor has mutations in any of the subunits of the SDH complex. Abnormal SDH immunostaining should be confirmed with germline genetic testing but patients invariable harbor germline mutations in one of the SDH subunits. Adrenal cortical carcinoma typically lacks this nested appearance, but there can be morphologic overlap. This tumor occurred outside the adrenal, but a panel of SF1, GATA3, and chromogranin should reliably distinguish pheochromocytoma/paraganglioma from adrenal cortical lesions should the need arise. Both entities typically lack keratin expression and are frequently synaptophysin positive so these stains are not useful in their distinction. Olfactory neuroblastoma shares a nested and lobulated architecture with paraganglioma and has a very similar immunoprofile – both express neuroendocrine markers and lack keratin expression, but the former entity is restricted to the area around the cribriform plate. Alveolar soft part sarcoma is an epithelioid, eosinophilic tumor but has the cytologic monotony associated with translocation tumors, lack expression of neuroendocrine markers and GATA3 and would harbor TFE3 rearrangement.
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Answer: E. Paraganglioma
Sections show an epithelioid neoplasm arranged in discrete small nests abutting the aorta. The cells are eosinophilic with speckled chromatin and there are numerous large multinucleated hyperchromatic cells. The morphology is the typical “zellballen” appearance of a paraganglioma, the term used for tumors arising from extra-adrenal autonomic tissue. This diagnosis is critical not to overlook because tumors often produce catecholamines leading to dangerous episodes of hypertension which may be exacerbated by surgical manipulation of the tumor. This entity has a very high correlation with germline genetic syndromes and genetic counseling is recommended for all patients. Screening for a subset of syndromes can be achieved with immunohistochemistry for SDH-B, which should be lost if the tumor has mutations in any of the subunits of the SDH complex. Abnormal SDH immunostaining should be confirmed with germline genetic testing but patients invariable harbor germline mutations in one of the SDH subunits. Adrenal cortical carcinoma typically lacks this nested appearance, but there can be morphologic overlap. This tumor occurred outside the adrenal, but a panel of SF1, GATA3, and chromogranin should reliably distinguish pheochromocytoma/paraganglioma from adrenal cortical lesions should the need arise. Both entities typically lack keratin expression and are frequently synaptophysin positive so these stains are not useful in their distinction. Olfactory neuroblastoma shares a nested and lobulated architecture with paraganglioma and has a very similar immunoprofile – both express neuroendocrine markers and lack keratin expression, but the former entity is restricted to the area around the cribriform plate. Alveolar soft part sarcoma is an epithelioid, eosinophilic tumor but has the cytologic monotony associated with translocation tumors, lack expression of neuroendocrine markers and GATA3 and would harbor TFE3 rearrangement.
Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen
Adult male with pelvic mass
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Adult male with pelvic mass
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Answer: C. Cellular angiofibroma
Histology: This tumor is well circumscribed and composed of uniform short spindled cells in a fibrous background.
Discussion: This entity, also known as angiomyofibroblastoma-like tumor of the male genital tract. These tumors are low grade and characterized by spindle cells with uniform oval nuclei with open chromatin and low to absent mitotic activity. Some of the spindle cells can show myoid differentiation and express SMA or desmin focally. There are numerous blood vessels some of which can have fibrin within the wall. CD34 is positive in most cases and ER/PR can be variable. The main differential diagnoses include schwannoma and neurofibroma which are positive for S100, while cellular angiofibromas are negative. Low grade fibromyxoid sarcoma could be in the differential diagnosis in biopsies, in which case MUC4 could assist in arriving at the right diagnosis. Perhaps the most critical differential diagnosis is aggressive angiomyxoma for which there is no immunohistochemistry that would be helpful. In contrast to cellular angiofibroma, aggressive angiomyxoma has a more myxoid background and an infiltrative border. Aggressive angiomyxoma requires complete surgical excision to prevent a recurrence.
References:
Am J Surg Pathol. January 1998 – Volume 22 – Issue 1 – p 6-16.
Incorrect
Answer: C. Cellular angiofibroma
Histology: This tumor is well circumscribed and composed of uniform short spindled cells in a fibrous background.
Discussion: This entity, also known as angiomyofibroblastoma-like tumor of the male genital tract. These tumors are low grade and characterized by spindle cells with uniform oval nuclei with open chromatin and low to absent mitotic activity. Some of the spindle cells can show myoid differentiation and express SMA or desmin focally. There are numerous blood vessels some of which can have fibrin within the wall. CD34 is positive in most cases and ER/PR can be variable. The main differential diagnoses include schwannoma and neurofibroma which are positive for S100, while cellular angiofibromas are negative. Low grade fibromyxoid sarcoma could be in the differential diagnosis in biopsies, in which case MUC4 could assist in arriving at the right diagnosis. Perhaps the most critical differential diagnosis is aggressive angiomyxoma for which there is no immunohistochemistry that would be helpful. In contrast to cellular angiofibroma, aggressive angiomyxoma has a more myxoid background and an infiltrative border. Aggressive angiomyxoma requires complete surgical excision to prevent a recurrence.
References:
Am J Surg Pathol. January 1998 – Volume 22 – Issue 1 – p 6-16.
Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen
Adult male with bladder tumor
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Adult male with bladder tumor
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Answer: A. Small cell carcinoma arising in urothelial carcinoma
Histology: Tumor shows extensive areas composed of small cells with scant cytoplasm, hyperchromatic nuclei with nuclear molding, inconspicuous nucleoli, frequent mitoses and numerous apoptotic bodies. There are areas of overlying carcinoma in-situ and foci of spindling of tumor cells.
Discussion: Small cell carcinoma arising in any site is a highly proliferative, high-grade neuroendocrine tumor. Small cell carcinoma of the bladder originates from urothelial cells through specific activation of the neuronal pathways. In support of this divergent differentiation pathway, small cell carcinoma of the bladder is frequently associated with conventional urothelial carcinoma and other histologic variants of urothelial carcinoma with molecular evidence of clonality. Guidelines support use of platinum and etoposide chemotherapy in the neoadjuvant and frontline settings akin to small cell carcinoma of lung. Radical cystectomy is a standard consolidative management strategy for the majority of patients with small cell carcinoma of the bladder with localized disease with/without regional lymphadenopathy. The appropriate identification of a small cell carcinoma component is critical to guide appropriate management. The differential diagnoses include rhabdomyosarcoma, lymphoma, and PNET. While rhabdomyosarcoma could be positive for synaptophysin, in contrast to small cell carcinoma they are negative for cytokeratin and show expression of desmin and/or myogenin. Small cell carcinomas are negative for lymphoid markers but can be focally positive for CD99 and NKX2.2, which are markers of PNET, but similarly to rhabdomyosarcoma, PNETs do not have significant expression of cytokeratin and are diffusely positive for CD99 or NKX2.2.
References:
Hum Pathol. 2018 Sep; 79: 57–65.
Incorrect
Answer: A. Small cell carcinoma arising in urothelial carcinoma
Histology: Tumor shows extensive areas composed of small cells with scant cytoplasm, hyperchromatic nuclei with nuclear molding, inconspicuous nucleoli, frequent mitoses and numerous apoptotic bodies. There are areas of overlying carcinoma in-situ and foci of spindling of tumor cells.
Discussion: Small cell carcinoma arising in any site is a highly proliferative, high-grade neuroendocrine tumor. Small cell carcinoma of the bladder originates from urothelial cells through specific activation of the neuronal pathways. In support of this divergent differentiation pathway, small cell carcinoma of the bladder is frequently associated with conventional urothelial carcinoma and other histologic variants of urothelial carcinoma with molecular evidence of clonality. Guidelines support use of platinum and etoposide chemotherapy in the neoadjuvant and frontline settings akin to small cell carcinoma of lung. Radical cystectomy is a standard consolidative management strategy for the majority of patients with small cell carcinoma of the bladder with localized disease with/without regional lymphadenopathy. The appropriate identification of a small cell carcinoma component is critical to guide appropriate management. The differential diagnoses include rhabdomyosarcoma, lymphoma, and PNET. While rhabdomyosarcoma could be positive for synaptophysin, in contrast to small cell carcinoma they are negative for cytokeratin and show expression of desmin and/or myogenin. Small cell carcinomas are negative for lymphoid markers but can be focally positive for CD99 and NKX2.2, which are markers of PNET, but similarly to rhabdomyosarcoma, PNETs do not have significant expression of cytokeratin and are diffusely positive for CD99 or NKX2.2.
Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen
Adult female with renal mass
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Adult female with renal mass
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Answer: C. Renal carcinoid
Histology: Well demarcated neoplasm composed of tightly packed cords and trabeculae of tumor cells with a ribbonlike appearance. The nuclei of tumor cells are elongated with their long axis perpendicular to the long axis of the cord.
Discussion: Primary renal carcinoid tumors are extremely rare and for an unknown reason, they have an association with horseshow kidney. They occur evenly divided between males and females, and most patients are diagnosed while asymptomatic and discovered to have metastasis at the time of diagnosis. The morphologic features of renal carcinoids are the same as carcinoid in other places and the most common architectural pattern is the one seen in this case with tightly packed cords of tumor cells. Mitoses range from 0/10HPFs to 3-4/10HPFs. By immunohistochemistry, renal carcinoids are positive for neuroendocrine markers synaptophysin and chromogranin and focally positive for CK7 or CK20 and negative for TTF-1 and WT1. The main differential diagnoses include other tumors with neuroendocrine differentiation such as small cell carcinoma, PNET, paraganglioma and neuroblastoma. Small cell carcinoma has high grade features that are not seen in carcinoid tumors including high mitotic activity, nuclear molding, and necrosis. Glandular structures seen in carcinoid tumors could simulate pseudorosettes seen in PNET but CD99 immunolabeling in PNET should help in that differential diagnosis. Neuroblastomas in the kidney are rare, especially in adults while primary carcinoids of the kidney have not been described yet in pediatric patients. Paragangliomas are negative for cytokeratin and contain a nested arrangement of tumor cells that are positive for GATA3.
References
Am J Surg Pathol. 2007 Oct;31(10):1539-44
J Surg Oncol. 1998;68:113–119.
Urology. 1976;8:146–148.
Incorrect
Answer: C. Renal carcinoid
Histology: Well demarcated neoplasm composed of tightly packed cords and trabeculae of tumor cells with a ribbonlike appearance. The nuclei of tumor cells are elongated with their long axis perpendicular to the long axis of the cord.
Discussion: Primary renal carcinoid tumors are extremely rare and for an unknown reason, they have an association with horseshow kidney. They occur evenly divided between males and females, and most patients are diagnosed while asymptomatic and discovered to have metastasis at the time of diagnosis. The morphologic features of renal carcinoids are the same as carcinoid in other places and the most common architectural pattern is the one seen in this case with tightly packed cords of tumor cells. Mitoses range from 0/10HPFs to 3-4/10HPFs. By immunohistochemistry, renal carcinoids are positive for neuroendocrine markers synaptophysin and chromogranin and focally positive for CK7 or CK20 and negative for TTF-1 and WT1. The main differential diagnoses include other tumors with neuroendocrine differentiation such as small cell carcinoma, PNET, paraganglioma and neuroblastoma. Small cell carcinoma has high grade features that are not seen in carcinoid tumors including high mitotic activity, nuclear molding, and necrosis. Glandular structures seen in carcinoid tumors could simulate pseudorosettes seen in PNET but CD99 immunolabeling in PNET should help in that differential diagnosis. Neuroblastomas in the kidney are rare, especially in adults while primary carcinoids of the kidney have not been described yet in pediatric patients. Paragangliomas are negative for cytokeratin and contain a nested arrangement of tumor cells that are positive for GATA3.
Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen
This is a solitary gastric mass in a patient with a distant history of melanoma.
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This is a solitary gastric mass in a patient with a distant history of melanoma.
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Answer: B. Gastrointestinal stromal tumor
Histologic Description: The morphology of this neoplasm closely resembles that of metastatic melanoma. The neoplastic cells are spindled and epithelioid, have focal intranuclear inclusions, and amphophilic cytoplasm. The neoplasm demonstrated immunoreactivity (focal) for Melan A, which furthers the overlap with melanoma. However, other melanoma markers such as HMB45 and S100 were negative. The neoplasm labeled diffusely for CD117 (which could also be seen in melanoma) but also for DOG1. Importantly, the neoplasm demonstrated loss of SDH immunoreactivity, supporting the diagnosis of a succinate dehydrogenase deficient gastrointestinal stromal tumor.
Differential Diagnosis: Melanoma should label for S100, SOX10, and/or HMB45, and would be SDH intact. Leiomyosarcoma would demonstrate immunoreactivity for muscle markers, and typically does not show the multinodular appearance seen in the current case. Paraganglioma would label for neuroendocrine markers such as synaptophysin and chromogranin. A subset of these would be SDH deficient, but paragangliomas should not typically label for CD117 or DOG1.
SDH deficient gastrointestinal stromal tumors differ from gastrointestinal stromal tumors in that they often affect children, are commonly multinodular, and associated with lymph node metastases. They have thus far been almost exclusively been reported in the stomach and their behavior is not well predicted by the conventional GIST criteria of size and mitosis.
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Answer: B. Gastrointestinal stromal tumor
Histologic Description: The morphology of this neoplasm closely resembles that of metastatic melanoma. The neoplastic cells are spindled and epithelioid, have focal intranuclear inclusions, and amphophilic cytoplasm. The neoplasm demonstrated immunoreactivity (focal) for Melan A, which furthers the overlap with melanoma. However, other melanoma markers such as HMB45 and S100 were negative. The neoplasm labeled diffusely for CD117 (which could also be seen in melanoma) but also for DOG1. Importantly, the neoplasm demonstrated loss of SDH immunoreactivity, supporting the diagnosis of a succinate dehydrogenase deficient gastrointestinal stromal tumor.
Differential Diagnosis: Melanoma should label for S100, SOX10, and/or HMB45, and would be SDH intact. Leiomyosarcoma would demonstrate immunoreactivity for muscle markers, and typically does not show the multinodular appearance seen in the current case. Paraganglioma would label for neuroendocrine markers such as synaptophysin and chromogranin. A subset of these would be SDH deficient, but paragangliomas should not typically label for CD117 or DOG1.
SDH deficient gastrointestinal stromal tumors differ from gastrointestinal stromal tumors in that they often affect children, are commonly multinodular, and associated with lymph node metastases. They have thus far been almost exclusively been reported in the stomach and their behavior is not well predicted by the conventional GIST criteria of size and mitosis.
Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen
This is a 60 year old female with a renal neoplasm.
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This is a 60 year old female with a renal neoplasm.
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Answer: C. Clear cell carcinoma and low grade oncocytic tumor (LOT) (collision)
Histologic Description: The lesion has two distinctive components. One is a hypervascular clear cell neoplasm which has the typical morphology of clear cell RCC, and demonstrates diffuse immunoreactivity for CA-IX. The second component labels not for CA-IX, but instead for cytokeratin 7 and not CD117. The neoplastic cells are epithelioid, and have slightly irregular nuclei with minimal with perinuclear halos, less well developed than those of chromophobe RCC. These are the typical features of low grade oncocytic tumor, a provisional entity which has been associated with TSC gene mutations.
Differential Diagnosis: High grade clear cell RCC typically would not demonstrate diffuse immunoreactivity for cytokeratin 7, and would have hypervascular stroma with greater nuclear atypia. Xp11 translocation RCC may have variable morphology, but does not demonstrate diffuse labeling for carbonic anhydrase IX and typically does not demonstrate the hypervascular stroma of the current clear cell lesion. Granular cell RCC is a historic term for high grade clear cell RCC which is no longer in use.
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Answer: C. Clear cell carcinoma and low grade oncocytic tumor (LOT) (collision)
Histologic Description: The lesion has two distinctive components. One is a hypervascular clear cell neoplasm which has the typical morphology of clear cell RCC, and demonstrates diffuse immunoreactivity for CA-IX. The second component labels not for CA-IX, but instead for cytokeratin 7 and not CD117. The neoplastic cells are epithelioid, and have slightly irregular nuclei with minimal with perinuclear halos, less well developed than those of chromophobe RCC. These are the typical features of low grade oncocytic tumor, a provisional entity which has been associated with TSC gene mutations.
Differential Diagnosis: High grade clear cell RCC typically would not demonstrate diffuse immunoreactivity for cytokeratin 7, and would have hypervascular stroma with greater nuclear atypia. Xp11 translocation RCC may have variable morphology, but does not demonstrate diffuse labeling for carbonic anhydrase IX and typically does not demonstrate the hypervascular stroma of the current clear cell lesion. Granular cell RCC is a historic term for high grade clear cell RCC which is no longer in use.
Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen
This is a 50 year old male with a history of colon cancer. A liver nodule was palpated and excised.
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This is a 50 year old male with a history of colon cancer. A liver nodule was palpated and excised.
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Answer: D. Focal nodular hyperplasia
Histologic Description: The morphology in this focus is essentially identical to that of cirrhosis. There is bridging fibrosis and regenerative nodules of hepatocytes. However, the lesion is a focal finding in an otherwise uninvolved liver. These are the typical features of focal nodular hyperplasia, which historically was once known as “focal cirrhosis”.
Differential Diagnosis: Cirrhosis would have similar histologic features as focal nodular hyperplasia, except it would diffusely involve the liver. Nodular transformation diffusely involves the liver and features regenerative nodules, but lacks bridging fibrosis. Congenital hepatic fibrosis is another diffuse process with bridging fibrosis, but lacks regenerative nodules.
Incorrect
Answer: D. Focal nodular hyperplasia
Histologic Description: The morphology in this focus is essentially identical to that of cirrhosis. There is bridging fibrosis and regenerative nodules of hepatocytes. However, the lesion is a focal finding in an otherwise uninvolved liver. These are the typical features of focal nodular hyperplasia, which historically was once known as “focal cirrhosis”.
Differential Diagnosis: Cirrhosis would have similar histologic features as focal nodular hyperplasia, except it would diffusely involve the liver. Nodular transformation diffusely involves the liver and features regenerative nodules, but lacks bridging fibrosis. Congenital hepatic fibrosis is another diffuse process with bridging fibrosis, but lacks regenerative nodules.
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen
A 55 year old male underwent a nephrectomy for a 2.4 cm renal mass.
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A 55 year old male underwent a nephrectomy for a 2.4 cm renal mass.
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Answer: B. Tubulocystic carcinoma
Histology: The lesion is well-circumscribed and unencapsulated without necrosis or hemorrhage. Microscopically, the tumor is composed of small to medium-sized cysts. A focal papillary component was present. Cytologically, there are large, round to polygonal cells with abundant eosinophilic cytoplasm. Cysts are lined by cells with hobnail appearance with vesicular nuclei and prominent nucleoli. Nuclei vary in shape, and some have irregular shapes.
Discussion: A variant of oncocytoma that can be confused with tubulocystic carcinoma is composed of tubules some with cystic dilatation. However, in addition, oncocytoma will typically also have some nests, which are lacking in tubulocystic carcinoma. In oncocytoma, one never see significant papillary component. Although both entities have dense Oncocytic cytoplasm, the nuclei in oncocytomas
are uniformly round with variably sized nucleoli. Cystic nephromas can have a similar architectural pattern although the cysts tend to be larger and lack the small cysts in tubulocystic carcinoma. Cystic nephromas are lined by cuboidal or hob nail epithelium and lack abundant oncocytic cytoplasm. ESC has more solid components and not just cysts lined by a single cell layer in tubulocystic carcinoma. Tubulocystic carcinomas are low grade with only uncommon metastases, despite having more prominent nucleoli, and should not be graded. In some tubulocystic RCCs there are poorly differentiated foci with solid area, areas resembling collecting duct carcinoma or prominent papillary carcinoma. In cases with poorly differentiated foci, the risk of metastases is significantly higher and a subset of these cases show low of fumarate hydratase (FH), typically sporadic unassociated with hereditary leiomyomatosis associated RCC (HLRCC).
Incorrect
Answer: B. Tubulocystic carcinoma
Histology: The lesion is well-circumscribed and unencapsulated without necrosis or hemorrhage. Microscopically, the tumor is composed of small to medium-sized cysts. A focal papillary component was present. Cytologically, there are large, round to polygonal cells with abundant eosinophilic cytoplasm. Cysts are lined by cells with hobnail appearance with vesicular nuclei and prominent nucleoli. Nuclei vary in shape, and some have irregular shapes.
Discussion: A variant of oncocytoma that can be confused with tubulocystic carcinoma is composed of tubules some with cystic dilatation. However, in addition, oncocytoma will typically also have some nests, which are lacking in tubulocystic carcinoma. In oncocytoma, one never see significant papillary component. Although both entities have dense Oncocytic cytoplasm, the nuclei in oncocytomas
are uniformly round with variably sized nucleoli. Cystic nephromas can have a similar architectural pattern although the cysts tend to be larger and lack the small cysts in tubulocystic carcinoma. Cystic nephromas are lined by cuboidal or hob nail epithelium and lack abundant oncocytic cytoplasm. ESC has more solid components and not just cysts lined by a single cell layer in tubulocystic carcinoma. Tubulocystic carcinomas are low grade with only uncommon metastases, despite having more prominent nucleoli, and should not be graded. In some tubulocystic RCCs there are poorly differentiated foci with solid area, areas resembling collecting duct carcinoma or prominent papillary carcinoma. In cases with poorly differentiated foci, the risk of metastases is significantly higher and a subset of these cases show low of fumarate hydratase (FH), typically sporadic unassociated with hereditary leiomyomatosis associated RCC (HLRCC).
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen
A 46 year old man underwent a nephrectomy for a 3.5 cm solid and cystic mass.
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A 46 year old man underwent a nephrectomy for a 3.5 cm solid and cystic mass.
Choose the correct diagnosis:
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Answer: B. Eosinophilic solid and cystic RCC (ESC RCC)
Microscopic description: The tumor is well circumscribed and non-encapsulated tumor with solid and cystic growth. The solid tumor areas show diffuse and compact nested growth. The cells have eosinophilic, voluminous cytoplasm, with numerous coarse amphophilic cytoplasmic granules (‘stippling’). The nuclei are round to oval, without prominent nucleoli. Cyst lining has a hobnail arrangement. Small clusters of admixed foamy histiocytes and lymphocytes are noted.
Discussion: ESC RCC is a recently described entity and the case shown here is classic for this entity. The great majority of ESC RCC are sporadic, but rare tumors with identical morphology have also been reported in patients with tuberous sclerosis. ESC RCC are typically solitary and low-stage tumors, but occasional multifocal and bilateral cases have been documented. Most are in females, with a broad age range, including pediatric patients. The vast majority of ESC RCC are indolent tumors, but rare cases have been reported with metastases. IHC reactivity for CK20 (either diffuse or focal) is present in about 85% of cases, with negative or very focally positive CK7. Molecular evaluation of sporadic ESC RCC by next generation sequencing (NGS) has demonstrated consistent and mutually exclusive, somatic bi-allelic mutations in the TSC genes, TSC1 and TSC2.
Incorrect
Answer: B. Eosinophilic solid and cystic RCC (ESC RCC)
Microscopic description: The tumor is well circumscribed and non-encapsulated tumor with solid and cystic growth. The solid tumor areas show diffuse and compact nested growth. The cells have eosinophilic, voluminous cytoplasm, with numerous coarse amphophilic cytoplasmic granules (‘stippling’). The nuclei are round to oval, without prominent nucleoli. Cyst lining has a hobnail arrangement. Small clusters of admixed foamy histiocytes and lymphocytes are noted.
Discussion: ESC RCC is a recently described entity and the case shown here is classic for this entity. The great majority of ESC RCC are sporadic, but rare tumors with identical morphology have also been reported in patients with tuberous sclerosis. ESC RCC are typically solitary and low-stage tumors, but occasional multifocal and bilateral cases have been documented. Most are in females, with a broad age range, including pediatric patients. The vast majority of ESC RCC are indolent tumors, but rare cases have been reported with metastases. IHC reactivity for CK20 (either diffuse or focal) is present in about 85% of cases, with negative or very focally positive CK7. Molecular evaluation of sporadic ESC RCC by next generation sequencing (NGS) has demonstrated consistent and mutually exclusive, somatic bi-allelic mutations in the TSC genes, TSC1 and TSC2.
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen
A 55 year old man presents with a 2.5 cm solid mass in the kidney. The tumor was diffusely positive for CK7 and negative for CD117 (Ckit).
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A 55 year old man presents with a 2.5 cm solid mass in the kidney. The tumor was diffusely positive for CK7 and negative for CD117 (Ckit).
Choose the correct diagnosis:
Correct
Answer: B. Low grade oncocytic tumor (LOT)
Microscopic Description: The tumor is circumscribed but unencapsulated neoplasm. Architecturally, it is composed of solid and compact nested growth. In the middle of the tumor is a sharply delineated loose stromal, edematous areas with loose reticular, cord-like and individual cells resembling tissue culture myofibroblasts. The cells have dense oncocytic cytoplasm with in general round nuclei with only focal nuclear irregularities. Focally, there were perinuclear halos or clearings. The lesions lacks significant cell atypia, pleomorphism, mitotic activity or
coagulative necrosis.
Discussion: Low-grade oncocytic tumor (LOT) is a recently proposed distinct renal entity, within the spectrum of oncocytic renal tumors. To date, there has been no malignant behavior, hence labeled as “tumor” as opposed to “carcinoma”. Additional follow up with larger series is needed before exact classification as either benign or low grade carcinoma is needed. In contrast to oncocytoma, the growth pattern is more diffuse compared to nests within a fibromyxoid background seen in oncocytoma. Whereas oncocytomas have round nuclei, LOTs also have a greater degree of nuclear irregularity, although typically focal. Diffuse CK7 staining also rules out oncocytoma, which are negative or shows only patchy staining. In contrast to chromophobe RCC, nuclei are not as crinkly (raisinoid) and LOTs lack the prominent cell borders typical of chromophobe RCC. Chromophobe RCC typically is CK7 and CD117 positive. A characteristic feature of LOT is the myxoid areas with cord-like growth and spindled cells.
Incorrect
Answer: B. Low grade oncocytic tumor (LOT)
Microscopic Description: The tumor is circumscribed but unencapsulated neoplasm. Architecturally, it is composed of solid and compact nested growth. In the middle of the tumor is a sharply delineated loose stromal, edematous areas with loose reticular, cord-like and individual cells resembling tissue culture myofibroblasts. The cells have dense oncocytic cytoplasm with in general round nuclei with only focal nuclear irregularities. Focally, there were perinuclear halos or clearings. The lesions lacks significant cell atypia, pleomorphism, mitotic activity or
coagulative necrosis.
Discussion: Low-grade oncocytic tumor (LOT) is a recently proposed distinct renal entity, within the spectrum of oncocytic renal tumors. To date, there has been no malignant behavior, hence labeled as “tumor” as opposed to “carcinoma”. Additional follow up with larger series is needed before exact classification as either benign or low grade carcinoma is needed. In contrast to oncocytoma, the growth pattern is more diffuse compared to nests within a fibromyxoid background seen in oncocytoma. Whereas oncocytomas have round nuclei, LOTs also have a greater degree of nuclear irregularity, although typically focal. Diffuse CK7 staining also rules out oncocytoma, which are negative or shows only patchy staining. In contrast to chromophobe RCC, nuclei are not as crinkly (raisinoid) and LOTs lack the prominent cell borders typical of chromophobe RCC. Chromophobe RCC typically is CK7 and CD117 positive. A characteristic feature of LOT is the myxoid areas with cord-like growth and spindled cells.
Presented by Dr. Ezra Baraban and prepared by Dr. Sintawat Wangsiricharoen.
A 20-year-old man with a testicular mass.
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A 20-year-old man with a testicular mass. Choose the correct diagnosis:
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Answer: B. Sclerosing Sertoli cell tumor.
Sections show a low grade neoplasm composed of cords and strands of bland cells within a dense collagenous stroma, between seminiferous tubules. The morphology does not fit with any established germ cell tumor subtype. The oval nuclei and delicate central nucleoli are typical of Sertoli cell tumor. The corded architecture raises adenomatoid tumor, but the entirely intratesticular location essentially excludes that diagnosis. The lack of speckled chromatin and dense collagenous stroma would be somewhat unusual for a carcinoid/neuroendocrine tumor, either primary or metastatic. Reactivity for SF1 would confirm sex-cord differentiation, and in combination with this morphology is diagnostic of a sclerosing sertoli cell tumor, a morphologic variant of sertoli cell tumor, which is divided into the NOS, sclerosing, large cell calcifying, and intratubular hyalinizing sertoli cell neoplasia categories.
Incorrect
Answer: B. Sclerosing Sertoli cell tumor.
Sections show a low grade neoplasm composed of cords and strands of bland cells within a dense collagenous stroma, between seminiferous tubules. The morphology does not fit with any established germ cell tumor subtype. The oval nuclei and delicate central nucleoli are typical of Sertoli cell tumor. The corded architecture raises adenomatoid tumor, but the entirely intratesticular location essentially excludes that diagnosis. The lack of speckled chromatin and dense collagenous stroma would be somewhat unusual for a carcinoid/neuroendocrine tumor, either primary or metastatic. Reactivity for SF1 would confirm sex-cord differentiation, and in combination with this morphology is diagnostic of a sclerosing sertoli cell tumor, a morphologic variant of sertoli cell tumor, which is divided into the NOS, sclerosing, large cell calcifying, and intratubular hyalinizing sertoli cell neoplasia categories.
Presented by Dr. Ezra Baraban and prepared by Dr. Sintawat Wangsiricharoen.
A 60-year-old man with a thigh mass.
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A 70-year-old man with a thigh mass. Choose the correct diagnosis:
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Answer: B. Epithelioid angiosarcoma.
Sections show a high grade malignant epithelioid neoplasm with focal prominent intercellular hemorrhage. The differential diagnosis is broad, and includes sarcomatoid carcinoma, melanoma, and various sarcomas. Negativity for S100 and SOX10 eliminate melanoma. Lack of primary carcinoma elsewhere and the absence of an epithelial component argue against sarcomatoid carcinoma. Expression of ERG, CD31, and CD34 support epithelioid angiosarcoma. Importantly, epithelioid sarcoma may express multiple endothelial markers, particularly CD34 and ERG. CD31 is typically negative, and importantly, INI1 expression will invariably be absent in epithelioid sarcoma and retained in epithelioid angiosarcoma.
Incorrect
Answer: B. Epithelioid angiosarcoma.
Sections show a high grade malignant epithelioid neoplasm with focal prominent intercellular hemorrhage. The differential diagnosis is broad, and includes sarcomatoid carcinoma, melanoma, and various sarcomas. Negativity for S100 and SOX10 eliminate melanoma. Lack of primary carcinoma elsewhere and the absence of an epithelial component argue against sarcomatoid carcinoma. Expression of ERG, CD31, and CD34 support epithelioid angiosarcoma. Importantly, epithelioid sarcoma may express multiple endothelial markers, particularly CD34 and ERG. CD31 is typically negative, and importantly, INI1 expression will invariably be absent in epithelioid sarcoma and retained in epithelioid angiosarcoma.
Presented by Dr. Ezra Baraban and prepared by Dr. Sintawat Wangsiricharoen.
A 77-year-old man with peritoneal carcinomatosis.
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A 77-year-old man with peritoneal carcinomatosis. Choose the correct diagnosis:
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Answer: C. Malignant mesothelioma.
Sections show an epithelioid neoplasm infiltrating adipose tissue, such that if this lesion were of mesothelial lineage, it would qualify as malignant mesothelioma. The main differential diagnosis is between metastatic adenocarcinoma and mesothelioma. Immunohistochemistry is critical, with positivity for multiple mesothelial markers (CK5/6, WT1, calretinin, and D240) and negativity for multiple carcinoma markers (claudin-4, MOC31, BerEP4) essential to support the mesothelial nature of this proliferation. Importantly, diffuse keratin expression is expected in mesothelial lesions, so cannot distinguish between adenocarcinoma and mesothelioma. If definitive invasion is not present, BAP1 or MTAP loss can support a diagnosis of mesothelioma, but retention of either of these does not exclude it. Desmoplastic small round cell tumor can have immunophenotypic overlap with mesothelioma (keratin, WT1 expression), but should lack calretinin and D240 expression, and would also typically label with desmin, and importantly, has an undifferentiated appearance morphologically, as opposed to the tubular morphology that predominates in this case. Rare mesotheliomas can have EWSR1 fusions, which are the hallmark of desmoplastic small round cell tumor, so the presence of an EWSR1 fusion alone does not distinguish between these two entities either.
Incorrect
Answer: C. Malignant mesothelioma.
Sections show an epithelioid neoplasm infiltrating adipose tissue, such that if this lesion were of mesothelial lineage, it would qualify as malignant mesothelioma. The main differential diagnosis is between metastatic adenocarcinoma and mesothelioma. Immunohistochemistry is critical, with positivity for multiple mesothelial markers (CK5/6, WT1, calretinin, and D240) and negativity for multiple carcinoma markers (claudin-4, MOC31, BerEP4) essential to support the mesothelial nature of this proliferation. Importantly, diffuse keratin expression is expected in mesothelial lesions, so cannot distinguish between adenocarcinoma and mesothelioma. If definitive invasion is not present, BAP1 or MTAP loss can support a diagnosis of mesothelioma, but retention of either of these does not exclude it. Desmoplastic small round cell tumor can have immunophenotypic overlap with mesothelioma (keratin, WT1 expression), but should lack calretinin and D240 expression, and would also typically label with desmin, and importantly, has an undifferentiated appearance morphologically, as opposed to the tubular morphology that predominates in this case. Rare mesotheliomas can have EWSR1 fusions, which are the hallmark of desmoplastic small round cell tumor, so the presence of an EWSR1 fusion alone does not distinguish between these two entities either.
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen.
A 65 year old man underwent resection of a paratesticular mass located at the base of the spermatic cord.
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A 65 year old man underwent resection of a paratesticular mass located at the base of the spermatic cord.
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Answer: C. Well-differentiated liposarcoma
Histology: The lesion was a soft lobulated, well-defined mass. It consisted primarily of irregular small bundles of benign-appearing smooth muscle with small foci of mature adipose tissue. Scattered cells with large atypical hyperchromatic, yet degenerative appearing, nuclei were present in the lesion.
Discussion: The location of this tumor is key. The vast majority of paratesticular soft tissue lesions in adults are liposarcoma. Leiomyosarcoma is the 2nd most common paratesticular mesenchymal tumor in adults. In contrast to this case, leiomyosarcoma consists of well-developed fascicles of cellular spindle cells with nuclear atypia and increased mitotic figures. If the lesion does not have the typical morphology of a leiomyosarcoma and is high grade and pleomorphic, then the likely diagnosis is de-differentiated liposarcoma and one should look at the benign-appearing adipose tissue for the large atypical smudgy hyperchromatic nuclei, typical of a well-differentiated component. Classic lipoblasts with indentation by intracytoplasmic lipid vacuoles leading to a characteristic scalloping of nuclear membrane are often not seen and not necessary for the diagnosis of well-differentiated liposarcoma. If the tumor is low grade, even if the adipose component is focal, again one should look for atypical cells and do MDM2 immunostaining to rule out a well-differentiated liposarcoma. This case is unusual due to the prominent smooth muscle component, which can uncommonly occur in well-differentiated liposarcoma. In the current case, MDM2 immunohistochemistry was positive verifying the diagnosis.
Reference: Folpe AL, Weiss SW. Lipoleiomyosarcoma (Well-Differentiated Liposarcoma With Leiomyosarcomatous Differentiation) A Clinicopathologic Study of Nine Cases Including One With Dedifferentiation. Am J Surg Pathol 2002; 26: 742-9.
Incorrect
Answer: C. Well-differentiated liposarcoma
Histology: The lesion was a soft lobulated, well-defined mass. It consisted primarily of irregular small bundles of benign-appearing smooth muscle with small foci of mature adipose tissue. Scattered cells with large atypical hyperchromatic, yet degenerative appearing, nuclei were present in the lesion.
Discussion: The location of this tumor is key. The vast majority of paratesticular soft tissue lesions in adults are liposarcoma. Leiomyosarcoma is the 2nd most common paratesticular mesenchymal tumor in adults. In contrast to this case, leiomyosarcoma consists of well-developed fascicles of cellular spindle cells with nuclear atypia and increased mitotic figures. If the lesion does not have the typical morphology of a leiomyosarcoma and is high grade and pleomorphic, then the likely diagnosis is de-differentiated liposarcoma and one should look at the benign-appearing adipose tissue for the large atypical smudgy hyperchromatic nuclei, typical of a well-differentiated component. Classic lipoblasts with indentation by intracytoplasmic lipid vacuoles leading to a characteristic scalloping of nuclear membrane are often not seen and not necessary for the diagnosis of well-differentiated liposarcoma. If the tumor is low grade, even if the adipose component is focal, again one should look for atypical cells and do MDM2 immunostaining to rule out a well-differentiated liposarcoma. This case is unusual due to the prominent smooth muscle component, which can uncommonly occur in well-differentiated liposarcoma. In the current case, MDM2 immunohistochemistry was positive verifying the diagnosis.
Reference: Folpe AL, Weiss SW. Lipoleiomyosarcoma (Well-Differentiated Liposarcoma With Leiomyosarcomatous Differentiation) A Clinicopathologic Study of Nine Cases Including One With Dedifferentiation. Am J Surg Pathol 2002; 26: 742-9.
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen.
A 23 year old man underwent an orchiectomy for testicular pain.
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A 23 year old man underwent an orchiectomy for testicular pain.
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Answer: C. Necrotic seminoma
Histology: A section of testis shows foci of cellular necrosis. Surrounding the necrosis there is prominent fibrosis with hemosiderin deposition and inflammation. Seminiferous tubules that are present appear atrophic with Sertoli only pattern and sclerosis.
Discussion: Occasional seminomas can undergo extensive necrosis, yet in the center of coagulative necrosis see ghosts of seminoma cells, as in this case. Even though the tumor is dead, one can make out ghosts of cells with vesicular nuclei with large central eosinophilic nucleoli, typical of seminoma. The adjacent testis with fibrosis, inflammation, and hemosiderin in this case likely represents a regressed tumor. In contrast with torsion, the necrosis involves the entire testis and it is coagulative necrosis where outlines of necrotic seminiferous tubules are visible. In an infarct, which is usually due to vasculitis, it is focal but also results in coagulative necrosis. In some cases adjacent to necrotic seminoma, atrophic seminiferous tubules containing germ cell neoplasia in-situ (GCNIS). Positive PLAP, CD117, and OCT4 may be maintained in necrotic seminoma, and these stains were focally faintly positive in this case. The stage can be variable and even entirely necrotic seminoma, some patients can present with metastatic disease.
Reference: Miller JS, Lee TL, Epstein JI, et al. The Utility of Microscopic Findings and Immunohistochemistry in the Classification of Necrotic Testicular Tumors: A Study of 11 Cases. Am J Surg Pathol 2009; 33: 1293-8.
Incorrect
Answer: C. Necrotic seminoma
Histology: A section of testis shows foci of cellular necrosis. Surrounding the necrosis there is prominent fibrosis with hemosiderin deposition and inflammation. Seminiferous tubules that are present appear atrophic with Sertoli only pattern and sclerosis.
Discussion: Occasional seminomas can undergo extensive necrosis, yet in the center of coagulative necrosis see ghosts of seminoma cells, as in this case. Even though the tumor is dead, one can make out ghosts of cells with vesicular nuclei with large central eosinophilic nucleoli, typical of seminoma. The adjacent testis with fibrosis, inflammation, and hemosiderin in this case likely represents a regressed tumor. In contrast with torsion, the necrosis involves the entire testis and it is coagulative necrosis where outlines of necrotic seminiferous tubules are visible. In an infarct, which is usually due to vasculitis, it is focal but also results in coagulative necrosis. In some cases adjacent to necrotic seminoma, atrophic seminiferous tubules containing germ cell neoplasia in-situ (GCNIS). Positive PLAP, CD117, and OCT4 may be maintained in necrotic seminoma, and these stains were focally faintly positive in this case. The stage can be variable and even entirely necrotic seminoma, some patients can present with metastatic disease.
Reference: Miller JS, Lee TL, Epstein JI, et al. The Utility of Microscopic Findings and Immunohistochemistry in the Classification of Necrotic Testicular Tumors: A Study of 11 Cases. Am J Surg Pathol 2009; 33: 1293-8.
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen.
A 55 year old man had a 3 cm cystic mass resected.
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A 55 year old man had a 3 cm cystic mass resected.
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Answer: A. Eosinophilic Solid and Cystic (ESC) RCC
Histology: The tumor was grossly and microscopically cystic with only small solid foci. The cysts were lined by cuboidal epithelium or hobnail cells with eosinophilic cytoplasm. The solid foci have similar cells with abundant eosinophilic cytoplasm. A minority of cells had prominent basophilic to purple cytoplasmic irregular granular stippling. Nuclei were round to oval, with often irregular nuclei. Focallly, cells had prominent nucleoli.
Discussion: ESC is a relatively newly described variant of RCC. It occurs most commonly in females with a broad age range, including pediatric patients. It is usually unifocal. The great majority are sporadic, but rare tumors with identical morphology have been reported in patients with tuberous sclerosis complex (TSC). There are somatic and bi-allelic mutations in the TSC genes, TSC1 and TSC2. Most cases are predominantly solid with focal cyst formation. However, at the other ends of the spectrums, some ESCs are very cystic as the case presented herein and some are purely solid. This case has the typical morphology with the exception of a more prominent cystic component. CK20 is present in almost all cases, either diffusely or focally, which is almost pathognomonic as other RCCs lack CK20. CK20 was positive in this case. Most cases are indolent with rare metastases. A grade is not assigned as they would in most cases be assigned a nucleolar grade 3, which would not reflect their biology.
Reference: Trpkov K, Williamson SR, Gill AJ, et al. Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia. Modern Pathology (in press).
Incorrect
Answer: A. Eosinophilic Solid and Cystic (ESC) RCC
Histology: The tumor was grossly and microscopically cystic with only small solid foci. The cysts were lined by cuboidal epithelium or hobnail cells with eosinophilic cytoplasm. The solid foci have similar cells with abundant eosinophilic cytoplasm. A minority of cells had prominent basophilic to purple cytoplasmic irregular granular stippling. Nuclei were round to oval, with often irregular nuclei. Focallly, cells had prominent nucleoli.
Discussion: ESC is a relatively newly described variant of RCC. It occurs most commonly in females with a broad age range, including pediatric patients. It is usually unifocal. The great majority are sporadic, but rare tumors with identical morphology have been reported in patients with tuberous sclerosis complex (TSC). There are somatic and bi-allelic mutations in the TSC genes, TSC1 and TSC2. Most cases are predominantly solid with focal cyst formation. However, at the other ends of the spectrums, some ESCs are very cystic as the case presented herein and some are purely solid. This case has the typical morphology with the exception of a more prominent cystic component. CK20 is present in almost all cases, either diffusely or focally, which is almost pathognomonic as other RCCs lack CK20. CK20 was positive in this case. Most cases are indolent with rare metastases. A grade is not assigned as they would in most cases be assigned a nucleolar grade 3, which would not reflect their biology.
Reference: Trpkov K, Williamson SR, Gill AJ, et al. Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia. Modern Pathology (in press).
Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen.
This is a 60-year-old female with a lung mass.
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This is a 60-year-old female with a lung mass.
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Answer: A. Adenocarcinoma, solid variant
Histologic Description: This lung tumor demonstrates well-formed acinar and focally papillary glandular areas at its periphery, which helps establish the diagnosis of adenocarcinoma. The difficulty with this case lies in the solid areas of the neoplasm. These areas form solid nests with well-developed cell borders, suggesting the possibility of an admixed squamous cell carcinoma component. This would make the tumor an adenosquamous carcinoma. Alternatively, the solid nested areas could represent a neuroendocrine component which would make this a combined neuroendocrine carcinoma, though the absence of neuroendocrine chromatin argues against that possibility. Focally, one can appreciate glands within the solid areas, and the cytology is similar to that of the well-formed glandular component. By immunohistochemistry, the solid area, like the glandular area, labels diffusely for TTF1 and for napsin A, but does not label for p40 or neuroendocrine markers like chromogranin and synaptophysin, supporting the diagnosis of solid variant adenocarcinoma.
Differential Diagnosis: Non-keratinizing squamous carcinoma is excluded by the absence of p40 labeling and presence of TTF1 labeling. The absence of neuroendocrine markers labeling argues against a combined neuroendocrine-adenocarcinoma, as does the absence of salt and pepper chromatin in the solid area.
This case illustrates how easy it is to mistake solid variant of adenocarcinoma for non-keratinizing squamous cell carcinoma in the lung. It is good practice to consider p40/TTF1 immunostains on non-keratinizing solid carcinomas in the lung before assigning squamous or glandular differentiation by morphology.
Incorrect
Answer: A. Adenocarcinoma, solid variant
Histologic Description: This lung tumor demonstrates well-formed acinar and focally papillary glandular areas at its periphery, which helps establish the diagnosis of adenocarcinoma. The difficulty with this case lies in the solid areas of the neoplasm. These areas form solid nests with well-developed cell borders, suggesting the possibility of an admixed squamous cell carcinoma component. This would make the tumor an adenosquamous carcinoma. Alternatively, the solid nested areas could represent a neuroendocrine component which would make this a combined neuroendocrine carcinoma, though the absence of neuroendocrine chromatin argues against that possibility. Focally, one can appreciate glands within the solid areas, and the cytology is similar to that of the well-formed glandular component. By immunohistochemistry, the solid area, like the glandular area, labels diffusely for TTF1 and for napsin A, but does not label for p40 or neuroendocrine markers like chromogranin and synaptophysin, supporting the diagnosis of solid variant adenocarcinoma.
Differential Diagnosis: Non-keratinizing squamous carcinoma is excluded by the absence of p40 labeling and presence of TTF1 labeling. The absence of neuroendocrine markers labeling argues against a combined neuroendocrine-adenocarcinoma, as does the absence of salt and pepper chromatin in the solid area.
This case illustrates how easy it is to mistake solid variant of adenocarcinoma for non-keratinizing squamous cell carcinoma in the lung. It is good practice to consider p40/TTF1 immunostains on non-keratinizing solid carcinomas in the lung before assigning squamous or glandular differentiation by morphology.
Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen.
This is a 40-year-old male with a large renal mass growing into the renal vein.
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This is a 40-year-old male with a large renal mass growing into the renal vein.
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Answer: A. Clear cell renal cell carcinoma
Histologic Description: This is a clear cell neoplasm with nested and papillary architecture. The cells have low grade nuclei, clear cytoplasm, and prominent subnuclear vacuolization, which suggest the diagnosis of clear cell papillary RCC. However, one can see on the section that the neoplastic cells are protruding into the renal sinus, and clinically were growing in the renal vein. Other portions of this tumor demonstrate solid acinar growth with a highly vascularized stroma with prominent septal capillaries, along with extruded blood and fibrin, which is much more typical of clear cell RCC. By immunohistochemistry, the neoplastic cells were diffusely positive for CA-IX and CD10, minimally positive for cytokeratin 7, and negative for TFE3 and cytokeratin 903. These results support the diagnosis of clear cell RCC which has areas mimicking clear cell papillary RCC.
Differential Diagnosis: Clear cell papillary RCC is typically an indolent neoplasm, and it would be very unusual for it to have renal vein involvement. Clear cell papillary RCC would label for cytokeratin 7 and would have a cup-shaped distribution of CA-IX labeling, and typically does not label much for CD10 but does label for cytokeratin 903. Xp11 translocation RCC (particularly those with the SFPQ or NONO gene TFE3 fusions) may closely mimic clear papillary RCC in that they demonstrate subnuclear vacuoles and clear cells with papillary architecture; however, these neoplasms should demonstrate TFE3 labeling, and should not show diffuse CA-IX labeling. Papillary RCC may have tubular areas, but would not have the water clear cytoplasm of the current lesions, and would label for cytokeratin 7.
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Answer: A. Clear cell renal cell carcinoma
Histologic Description: This is a clear cell neoplasm with nested and papillary architecture. The cells have low grade nuclei, clear cytoplasm, and prominent subnuclear vacuolization, which suggest the diagnosis of clear cell papillary RCC. However, one can see on the section that the neoplastic cells are protruding into the renal sinus, and clinically were growing in the renal vein. Other portions of this tumor demonstrate solid acinar growth with a highly vascularized stroma with prominent septal capillaries, along with extruded blood and fibrin, which is much more typical of clear cell RCC. By immunohistochemistry, the neoplastic cells were diffusely positive for CA-IX and CD10, minimally positive for cytokeratin 7, and negative for TFE3 and cytokeratin 903. These results support the diagnosis of clear cell RCC which has areas mimicking clear cell papillary RCC.
Differential Diagnosis: Clear cell papillary RCC is typically an indolent neoplasm, and it would be very unusual for it to have renal vein involvement. Clear cell papillary RCC would label for cytokeratin 7 and would have a cup-shaped distribution of CA-IX labeling, and typically does not label much for CD10 but does label for cytokeratin 903. Xp11 translocation RCC (particularly those with the SFPQ or NONO gene TFE3 fusions) may closely mimic clear papillary RCC in that they demonstrate subnuclear vacuoles and clear cells with papillary architecture; however, these neoplasms should demonstrate TFE3 labeling, and should not show diffuse CA-IX labeling. Papillary RCC may have tubular areas, but would not have the water clear cytoplasm of the current lesions, and would label for cytokeratin 7.
Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen.
This is a 63-year-old male who presents with brain metastasis and a known large lung mass.
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This is a 63-year-old male who presents with brain metastasis and a known large lung mass.
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Answer: C. SMARCA4 deficient carcinoma
Histologic Description: This is a high grade neoplasm composed of sheets of malignant epithelioid cells. Focally, there are areas that suggest gland formation, but the architecture is mainly solid. The neoplastic cells have vesicular chromatin and prominent nucleoli, and eccentric pink cytoplasm, giving a slightly rhabdoid or hepatoid appearance. There are numerous associated inflammatory cells, mainly lymphocytes with occasional neutrophils. The neoplastic cells are diffusely positive for cytokeratin A1/3. They demonstrate focal labeling for p40 and patchy labeling CK5/6, but are negative for TTF1 and napsin A. Neoplastic cells demonstrate loss of SMARCA4 protein, supporting the diagnosis of SMAR A4 deficient adenocarcinoma metastatic from the lung.
Differential Diagnosis: Poorly differentiated squamous cell carcinoma is a significant consideration giving the focal labeling for p40 and patchy CK5/6 labeling. However, the rhabdoid and hepatoid morphology of the neoplastic cells would not be typical of poorly differentiated squamous cell carcinomas, which are often basaloid. Poorly differentiated adenocarcinoma is suggested by the prominent nucleoli and vesicular chromatin, but the absence of TTF1 and napsin A staining argues against a typical lung adenocarcinoma. ALK-rearranged lung adenocarcinomas often have mucinous cribriform morphology and would label for ALK protein.
Morphologic clues to a SMARCA4 deficient lung carcinoma include variable cell size but absence of severe pleomorphism, hepatoid or rhabdoid morphology, admixed neutrophils, and TTF1 negativity with glandular morphology.
Incorrect
Answer: C. SMARCA4 deficient carcinoma
Histologic Description: This is a high grade neoplasm composed of sheets of malignant epithelioid cells. Focally, there are areas that suggest gland formation, but the architecture is mainly solid. The neoplastic cells have vesicular chromatin and prominent nucleoli, and eccentric pink cytoplasm, giving a slightly rhabdoid or hepatoid appearance. There are numerous associated inflammatory cells, mainly lymphocytes with occasional neutrophils. The neoplastic cells are diffusely positive for cytokeratin A1/3. They demonstrate focal labeling for p40 and patchy labeling CK5/6, but are negative for TTF1 and napsin A. Neoplastic cells demonstrate loss of SMARCA4 protein, supporting the diagnosis of SMAR A4 deficient adenocarcinoma metastatic from the lung.
Differential Diagnosis: Poorly differentiated squamous cell carcinoma is a significant consideration giving the focal labeling for p40 and patchy CK5/6 labeling. However, the rhabdoid and hepatoid morphology of the neoplastic cells would not be typical of poorly differentiated squamous cell carcinomas, which are often basaloid. Poorly differentiated adenocarcinoma is suggested by the prominent nucleoli and vesicular chromatin, but the absence of TTF1 and napsin A staining argues against a typical lung adenocarcinoma. ALK-rearranged lung adenocarcinomas often have mucinous cribriform morphology and would label for ALK protein.
Morphologic clues to a SMARCA4 deficient lung carcinoma include variable cell size but absence of severe pleomorphism, hepatoid or rhabdoid morphology, admixed neutrophils, and TTF1 negativity with glandular morphology.
Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Sintawat Wangsiricharoen
35 year-old female with a breast mass
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35-year-old female with a breast mass
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Diagnosis: A. Fibromatosis.
Histology:
The stellate tumor is composed of bland spindle cells with no nuclear atypia nor mitotic activity. The capillaries within the lesion appear to “stand out” at low power because the lesional cells are hypochromatic relative to the endothelial nuclei, and there is perivascular clearing leading to a small gap between the blood vessels and the lesional spindle cells. There are scattered lymphoid aggregates within and adjacent to the tumor. Malignant epithelial elements or leaf-like architecture is not identified. The lesional spindle cells are negative for cytokeratin labeling and display nuclear beta-catenin labeling.
Discussion:
This lesion is a fibromatosis, or extra-abdominal desmoid tumor, involving the breast. Breast fibromatoses are histologically and immunphenotypically identical to fibromatoses elsewhere in the body. The leading differential diagnoses are spindle cell metaplastic carcinoma (low-grade fibromatosis-like spindle cell carcinoma), scar, and nodular fasciitis. Spindle cell metaplastic carcinoma will be immunoreactive for cytokeratins, although not necessarily for every cytokeratin antibody, whereas fibromatosis is negative for cytokeratin. Nuclear-beta catenin labeling is seen in approximately 75% of fibromatoses and labeling supports the histologic impression of fibromatosis…however! Nuclear beta-catenin labeling can be seen in a minority of both malignant phyllodes tumors and spindle cell metaplastic carcinomas, such that cytokeratin must always be performed on a potential fibromatosis in the breast in order to exclude spindle cell carcinoma.
References:
Charu V, Cimino-Mathews A. Spindle cell lesions of the breast. Am J Surg Pathol: Reviews & Reports. 2017 Mar-Apr;22(2):116-124
Incorrect
Diagnosis: A. Fibromatosis.
Histology:
The stellate tumor is composed of bland spindle cells with no nuclear atypia nor mitotic activity. The capillaries within the lesion appear to “stand out” at low power because the lesional cells are hypochromatic relative to the endothelial nuclei, and there is perivascular clearing leading to a small gap between the blood vessels and the lesional spindle cells. There are scattered lymphoid aggregates within and adjacent to the tumor. Malignant epithelial elements or leaf-like architecture is not identified. The lesional spindle cells are negative for cytokeratin labeling and display nuclear beta-catenin labeling.
Discussion:
This lesion is a fibromatosis, or extra-abdominal desmoid tumor, involving the breast. Breast fibromatoses are histologically and immunphenotypically identical to fibromatoses elsewhere in the body. The leading differential diagnoses are spindle cell metaplastic carcinoma (low-grade fibromatosis-like spindle cell carcinoma), scar, and nodular fasciitis. Spindle cell metaplastic carcinoma will be immunoreactive for cytokeratins, although not necessarily for every cytokeratin antibody, whereas fibromatosis is negative for cytokeratin. Nuclear-beta catenin labeling is seen in approximately 75% of fibromatoses and labeling supports the histologic impression of fibromatosis…however! Nuclear beta-catenin labeling can be seen in a minority of both malignant phyllodes tumors and spindle cell metaplastic carcinomas, such that cytokeratin must always be performed on a potential fibromatosis in the breast in order to exclude spindle cell carcinoma.
References:
Charu V, Cimino-Mathews A. Spindle cell lesions of the breast. Am J Surg Pathol: Reviews & Reports. 2017 Mar-Apr;22(2):116-124
Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Sintawat Wangsiricharoen
50-year-old female with breast mass
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50 year-old female with breast mass
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Diagnosis: B. Malignant phyllodes tumor
Histology:
The tumor is composed of markedly atypical epithelioid spindle cells with regions of fascicular architecture, areas of stromal myxoid change, and variable cellularity, including abrupt transition from hypercellular to hypocellular areas. There is brisk mitotic activity. Malignant epithelial areas are not identified. At one end of the section, hyalinized leaf-like projections are identified; they are ischemic and devoid of epithelial lining.
Discussion:
This lesion is a malignant phyllodes tumor of the breast, characterized here by extensive stromal overgrowth (defined as one 4X low power field entirely composed of stroma), markedly atypical stromal cells with brisk mitotic activity, and focal leaf-like architecture. Phyllodes tumors are rare fibroepithelial lesions of the breast and are subdivided into benign, borderline, and malignant classification. The diagnosis of malignant phyllodes tumor can be challenging due to the often extensive stromal overgrowth and paucity or absence of the benign epithelial component. The presence of a leaf-like architecture and/or an associated lower grade fibroepithelial lesion makes it possible to diagnose a malignant phyllodes tumor on the H&E alone. The differential diagnosis includes metaplastic carcinoma, metaplastic carcinoma, and metaplastic carcinoma, followed by primary or metastatic sarcoma to the breast. The most useful diagnostic tool is to take additional tissue sections to assess for tumoral heterogeneity and the possibility of a focal leaf-like component. A subset of malignant phyllodes tumors are immunoreactive for CD34, which is negative in metaplastic carcinomas and can be a useful diagnostic tool if positive.
Reference:
Tan BY, Acs G, Apple SK, et al. Phyllodes tumours of the breast: a consensus review. Histopathology. 2016 Jan;68(1):5-21.
Incorrect
Diagnosis: B. Malignant phyllodes tumor
Histology:
The tumor is composed of markedly atypical epithelioid spindle cells with regions of fascicular architecture, areas of stromal myxoid change, and variable cellularity, including abrupt transition from hypercellular to hypocellular areas. There is brisk mitotic activity. Malignant epithelial areas are not identified. At one end of the section, hyalinized leaf-like projections are identified; they are ischemic and devoid of epithelial lining.
Discussion:
This lesion is a malignant phyllodes tumor of the breast, characterized here by extensive stromal overgrowth (defined as one 4X low power field entirely composed of stroma), markedly atypical stromal cells with brisk mitotic activity, and focal leaf-like architecture. Phyllodes tumors are rare fibroepithelial lesions of the breast and are subdivided into benign, borderline, and malignant classification. The diagnosis of malignant phyllodes tumor can be challenging due to the often extensive stromal overgrowth and paucity or absence of the benign epithelial component. The presence of a leaf-like architecture and/or an associated lower grade fibroepithelial lesion makes it possible to diagnose a malignant phyllodes tumor on the H&E alone. The differential diagnosis includes metaplastic carcinoma, metaplastic carcinoma, and metaplastic carcinoma, followed by primary or metastatic sarcoma to the breast. The most useful diagnostic tool is to take additional tissue sections to assess for tumoral heterogeneity and the possibility of a focal leaf-like component. A subset of malignant phyllodes tumors are immunoreactive for CD34, which is negative in metaplastic carcinomas and can be a useful diagnostic tool if positive.
Reference:
Tan BY, Acs G, Apple SK, et al. Phyllodes tumours of the breast: a consensus review. Histopathology. 2016 Jan;68(1):5-21.
Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Sintawat Wangsiricharoen
60-year-old female with a breast mass
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60 year-old female with a breast mass
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Diagnosis: C. Metaplastic carcinoma
Histology:
The tumor is composed atypical spindle cells with variable cellularity; hypercellular regions alternate with hypocellular regions, the latter of which displays expanded, dense, eosinophilic collagen. Within the hypercellular regions, there are also small bands of the brightly eosinophilic, dense collagen. At the ends of the tissue sections, foci of malignant squamous cell carcinoma are identified. eaf-like architecture are seen.
Discussion:
This lesion is a metaplastic carcinoma of the breast, characterized in this case by intermediate-grade malignant spindle cells admixed with squamous cell carcinoma. Metaplastic carcinomas of the breast are more common than phyllodes tumors, which are more common than metastatic sarcoma to the breast or primary breast sarcomas. The presence of a malignant epithelial component (i.e., the squamous cell carcinoma) enables making the diagnosis of metaplastic carcinoma on the basis of the H&E sections. This malignant epithelial differentiation can be focal. Immunostains for cytokeratins such as AE1/AE3, CK903, and CAM5.2 can be used to highlight epithelial differentiation within the malignant spindle cells; however, cytokeratin labeling can be seen in malignant phyllodes tumor, so focal cytokeratin labeling alone in malignant spindle cells is not enough to differentiate a metaplastic carcinoma from a malignant phyllodes tumor. In the absence of the malignant epithelial component, the most useful next step is often to take additional tumor sections to look for an unsampled epithelial component.
Reference:
Charu V, Cimino-Mathews A. Spindle cell lesions of the breast. Am J Surg Pathol: Reviews & Reports. 2017 Mar-Apr;22(2):116-124
Incorrect
Diagnosis: C. Metaplastic carcinoma
Histology:
The tumor is composed atypical spindle cells with variable cellularity; hypercellular regions alternate with hypocellular regions, the latter of which displays expanded, dense, eosinophilic collagen. Within the hypercellular regions, there are also small bands of the brightly eosinophilic, dense collagen. At the ends of the tissue sections, foci of malignant squamous cell carcinoma are identified. eaf-like architecture are seen.
Discussion:
This lesion is a metaplastic carcinoma of the breast, characterized in this case by intermediate-grade malignant spindle cells admixed with squamous cell carcinoma. Metaplastic carcinomas of the breast are more common than phyllodes tumors, which are more common than metastatic sarcoma to the breast or primary breast sarcomas. The presence of a malignant epithelial component (i.e., the squamous cell carcinoma) enables making the diagnosis of metaplastic carcinoma on the basis of the H&E sections. This malignant epithelial differentiation can be focal. Immunostains for cytokeratins such as AE1/AE3, CK903, and CAM5.2 can be used to highlight epithelial differentiation within the malignant spindle cells; however, cytokeratin labeling can be seen in malignant phyllodes tumor, so focal cytokeratin labeling alone in malignant spindle cells is not enough to differentiate a metaplastic carcinoma from a malignant phyllodes tumor. In the absence of the malignant epithelial component, the most useful next step is often to take additional tumor sections to look for an unsampled epithelial component.
Reference:
Charu V, Cimino-Mathews A. Spindle cell lesions of the breast. Am J Surg Pathol: Reviews & Reports. 2017 Mar-Apr;22(2):116-124
Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen.
Adult female with kidney tumor.
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Adult female with kidney tumor.
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Correct: B.
Histology: This tumor is composed of a spindle cells with abundant light eosinophilic cytoplasm, arranged in a patternless pattern. There are foci of significant nuclear pleomorphism.
Discussion: Renal angiomyolipomas (AMLs) are tumors formed of variable amounts of three components: adipose tissue, tortuous thick-walled vessels and cells with smooth muscle differentiation. There is evidence that the AMLs belong to the family of perivascular epithelioid cell tumors (PEComas) It can be associated with tuberous sclerosis but they are most frequently sporadic. Sporadic AMLs are 4 times more frequent in women. The clinical presentation is similar to other renal masses: incidental finding, abdominal pain and/or hematuria. A minority of patients may present with rupture and intrabdominal bleeding. Grossly, AMLs are typically well-circumscribed, sometimes with infiltrating edges. It may involve the perirenal soft tissue and even lymph nodes but these features are not associated with malignancy. Histologically, AMLs show vessels with thick wall and spindle cells radiating off the wall. The spindle cells have an appearance similar to smooth muscle cells with blunt end elongated nuclei and eosinophilic cytoplasm. Variants of AMLs include fat poor, epithelioid and epithelioid AML with atypia, and angiomyolipoma with epithelial cysts. Fat poor-AMLs are more common in needle biopsy specimens because they are difficult to distinguish from renal cell carcinoma on image studies and, therefore, undergo more frequent biopsy. The epithelioid variant is characterized by round cells with variable amount of eosinophilic or clear cytoplasm and sometimes bizarre nuclear atypia (atypical epithelioid AMLs). Rarely, epithelioid AMLs can have a malignant behavior. Malignancy in AMLs has been associated with the presence of at least 3 of the following findings: 1) >70% atypical epithelioid cells, 2) 2 or more mitotic figures per 10 HPFs, 3) atypical mitotic figures, 4) necrosis. By immunohistochemistry, AMLs are positive for melanocytic markers HMB45, Melan-A and tyrosinase. These are often focally positive and therefore a panel of markers is recommended. Other positive markers include cathepsin-K, and smooth muscle actin. PAX-8 and keratin stains are frequently negative and might be helpful in the distinction of epithelioid AMLs and renal cell carcinoma
References:
1. Eble JN. Angiomyolipoma of kidney. Semin Diagn Pathol. 1998;15(1):21-40.
2. Lin C, Jin L, Yang Y, Ding Y, Wu X, Ni L, et al. Tuberous sclerosis-associated renal angiomyolipoma: A report of two cases and review of the literature. Mol Clin Oncol. 2017;7(4):706-8.
3. Nese N, Martignoni G, Fletcher CD, Gupta R, Pan CC, Kim H, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011;35(2):161-76.
4. Brimo F, Robinson B, Guo C, Zhou M, Latour M, Epstein JI. Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol. 2010;34(5):715-22.
5. Fine SW, Reuter VE, Epstein JI, Argani P. Angiomyolipoma with epithelial cysts (AMLEC): a distinct cystic variant of angiomyolipoma. Am J Surg Pathol. 2006;30(5):593-9.
6. Park BK. Renal Angiomyolipoma: Radiologic Classification and Imaging Features According to the Amount of Fat. AJR Am J Roentgenol. 2017;209(4):826-35.
Incorrect
Correct: B.
Histology: This tumor is composed of a spindle cells with abundant light eosinophilic cytoplasm, arranged in a patternless pattern. There are foci of significant nuclear pleomorphism.
Discussion: Renal angiomyolipomas (AMLs) are tumors formed of variable amounts of three components: adipose tissue, tortuous thick-walled vessels and cells with smooth muscle differentiation. There is evidence that the AMLs belong to the family of perivascular epithelioid cell tumors (PEComas) It can be associated with tuberous sclerosis but they are most frequently sporadic. Sporadic AMLs are 4 times more frequent in women. The clinical presentation is similar to other renal masses: incidental finding, abdominal pain and/or hematuria. A minority of patients may present with rupture and intrabdominal bleeding. Grossly, AMLs are typically well-circumscribed, sometimes with infiltrating edges. It may involve the perirenal soft tissue and even lymph nodes but these features are not associated with malignancy. Histologically, AMLs show vessels with thick wall and spindle cells radiating off the wall. The spindle cells have an appearance similar to smooth muscle cells with blunt end elongated nuclei and eosinophilic cytoplasm. Variants of AMLs include fat poor, epithelioid and epithelioid AML with atypia, and angiomyolipoma with epithelial cysts. Fat poor-AMLs are more common in needle biopsy specimens because they are difficult to distinguish from renal cell carcinoma on image studies and, therefore, undergo more frequent biopsy. The epithelioid variant is characterized by round cells with variable amount of eosinophilic or clear cytoplasm and sometimes bizarre nuclear atypia (atypical epithelioid AMLs). Rarely, epithelioid AMLs can have a malignant behavior. Malignancy in AMLs has been associated with the presence of at least 3 of the following findings: 1) >70% atypical epithelioid cells, 2) 2 or more mitotic figures per 10 HPFs, 3) atypical mitotic figures, 4) necrosis. By immunohistochemistry, AMLs are positive for melanocytic markers HMB45, Melan-A and tyrosinase. These are often focally positive and therefore a panel of markers is recommended. Other positive markers include cathepsin-K, and smooth muscle actin. PAX-8 and keratin stains are frequently negative and might be helpful in the distinction of epithelioid AMLs and renal cell carcinoma
References:
1. Eble JN. Angiomyolipoma of kidney. Semin Diagn Pathol. 1998;15(1):21-40.
2. Lin C, Jin L, Yang Y, Ding Y, Wu X, Ni L, et al. Tuberous sclerosis-associated renal angiomyolipoma: A report of two cases and review of the literature. Mol Clin Oncol. 2017;7(4):706-8.
3. Nese N, Martignoni G, Fletcher CD, Gupta R, Pan CC, Kim H, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011;35(2):161-76.
4. Brimo F, Robinson B, Guo C, Zhou M, Latour M, Epstein JI. Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol. 2010;34(5):715-22.
5. Fine SW, Reuter VE, Epstein JI, Argani P. Angiomyolipoma with epithelial cysts (AMLEC): a distinct cystic variant of angiomyolipoma. Am J Surg Pathol. 2006;30(5):593-9.
6. Park BK. Renal Angiomyolipoma: Radiologic Classification and Imaging Features According to the Amount of Fat. AJR Am J Roentgenol. 2017;209(4):826-35.
Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen.
Female in her 20s with kidney mass.
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Female in her 20s with kidney mass.
Correct
Correct: C
Histology: The tumor is composed of back-to-back nests with tumor cells with round nuclei, inconspicuous nucleoli, “neuroendocrine” appearing chromatin, and moderate amount of eosinophilic cytoplasm with occasional eosinophilic cytoplasmic inclusions.
Discussion: SDH is an enzymatic complex localized within the mitochondria and composed of multiple subunits. Mutation in any one gene that codes for the proteins of each subunit can lead to absence of SDHB by immunohistochemistry. Studies show that SDH immunohistochemistry deficiency highly correlates (more than 90% of cases) with germline mutation. Patients with SDHB negative tumors should undergo genetic counseling.
Histology: The tumor is composed of back-to-back nests with tumor cells with round nuclei, inconspicuous nucleoli, “neuroendocrine” appearing chromatin, and moderate amount of eosinophilic cytoplasm with occasional eosinophilic cytoplasmic inclusions.
Discussion: SDH is an enzymatic complex localized within the mitochondria and composed of multiple subunits. Mutation in any one gene that codes for the proteins of each subunit can lead to absence of SDHB by immunohistochemistry. Studies show that SDH immunohistochemistry deficiency highly correlates (more than 90% of cases) with germline mutation. Patients with SDHB negative tumors should undergo genetic counseling.
Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen.
Adult male with paratesticular mass.
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Case 1. Adult male with paratesticular mass.
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Correct: D
Histology: Proliferation of cords of tumor cells forming tubules, with cytoplasmic vacuoles and surrounded by prominent smooth muscle.
Discussion: Adenomatoid tumors present as a scrotal mass and are usually suspected by image studies and therefore frequently excised and submitted for frozen section by the surgeon with the goal to avoid a complete orchiectomy. Tumor cells have bland nuclei with open chromatin and arranged in cords or forming tubules and show intracytoplasmic clear vacuoles. The surrounding stroma often shows prominent smooth muscle differentiation and acquire a more reactive appearance in cases with infarction. While these tumors are most frequently paratesticular, they can occasionally involve testicular parenchyma. By immunohistochemistry, they are positive for mesothelial markers including calretinin and WT1, but are negative for SF1, which can be used in cases difficult to differentiate from Sertoli cell tumors.
References:
Urol Case Rep. 2018 Nov; 21: 34–35.
Int J Clin Exp Pathol. 2015; 8(5): 5914–5918.
Scientific World Journal. 2004; 4: 11–15.
Incorrect
Correct: D
Histology: Proliferation of cords of tumor cells forming tubules, with cytoplasmic vacuoles and surrounded by prominent smooth muscle.
Discussion: Adenomatoid tumors present as a scrotal mass and are usually suspected by image studies and therefore frequently excised and submitted for frozen section by the surgeon with the goal to avoid a complete orchiectomy. Tumor cells have bland nuclei with open chromatin and arranged in cords or forming tubules and show intracytoplasmic clear vacuoles. The surrounding stroma often shows prominent smooth muscle differentiation and acquire a more reactive appearance in cases with infarction. While these tumors are most frequently paratesticular, they can occasionally involve testicular parenchyma. By immunohistochemistry, they are positive for mesothelial markers including calretinin and WT1, but are negative for SF1, which can be used in cases difficult to differentiate from Sertoli cell tumors.
References:
Urol Case Rep. 2018 Nov; 21: 34–35.
Int J Clin Exp Pathol. 2015; 8(5): 5914–5918.
Scientific World Journal. 2004; 4: 11–15.
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen
A 65 year old man underwent an enucleation for urinary obstructive symptoms.
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A 65 year old man underwent an enucleation for urinary obstructive symptoms.
Correct
Answer: A
Histological Description: The enucleation consisted of a pure stromal proliferation with focal infarction. The lesion has relatively low cellularity with no pleomorphism or mitotic activity.
Discussion: There are five patterns of STUMP
Most common pattern with slightly hypercellular or normocellular stroma with scattered atypical, but degenerative appearing cells glands. Glands can be crowded with basal cell hyperplasia, adenosis, cribriform hyperplasia, squamous and urothelial metaplasia. Does not mimic BPH.
2nd pattern with hypercellular stroma consisting of bland fusiform stromal cells with eosinophilic cytoplasm admixed with benign glands.
3rd pattern with myxoid stroma containing bland stromal cells and often lacking admixed glands in sheets of stroma without nodularity. Lacks thick walled arterioles.
4th pattern is phyllodes with leaf-like hypocellular fibrous stroma covered by benign appearing prostatic epithelium. Can have associated complex glandular proliferations of basal cell hyperplasia, adenosis, cribriform hyperplasia, squamous and urothelial metaplasia.
5th pattern is round cell with increased stromal cellularity composed of cells with round bland nuclei. In contrast, stromal BPH nuclei are oval and spindled. Can have associated complex glandular proliferations. Abrupt demarcation between normal stroma and STUMP.
The 2nd pattern of STUMP most closely resembles stromal nodular BPH. BPH stroma is not as cellular as 2nd pattern of STUMP and lacks eosinophilic cytoplasm. Also in contrast to STUMP, BPH stroma has nodularity and numerous thick walled small arterioles. These vessels are distinctive, as typically such small vessels would not have thick walls. This feature is particularly helpful on needle biopsy, where the multinodularity cannot be appreciated. This case further mimicked a neoplasm with the presence of necrosis. BPH, stromal or glandular-stromal can undergo infarction which correlates with size of BPH and also systemic atherosclerosis. The
current case lacks the marked hypercellularity, pleomorphism, and mitotic activity seen in stromal sarcomas. Stromal nodular BPH may be extensive without any prostate glands mimicking a stroma neoplasm.
Incorrect
Answer: A
Histological Description: The enucleation consisted of a pure stromal proliferation with focal infarction. The lesion has relatively low cellularity with no pleomorphism or mitotic activity.
Discussion: There are five patterns of STUMP
Most common pattern with slightly hypercellular or normocellular stroma with scattered atypical, but degenerative appearing cells glands. Glands can be crowded with basal cell hyperplasia, adenosis, cribriform hyperplasia, squamous and urothelial metaplasia. Does not mimic BPH.
2nd pattern with hypercellular stroma consisting of bland fusiform stromal cells with eosinophilic cytoplasm admixed with benign glands.
3rd pattern with myxoid stroma containing bland stromal cells and often lacking admixed glands in sheets of stroma without nodularity. Lacks thick walled arterioles.
4th pattern is phyllodes with leaf-like hypocellular fibrous stroma covered by benign appearing prostatic epithelium. Can have associated complex glandular proliferations of basal cell hyperplasia, adenosis, cribriform hyperplasia, squamous and urothelial metaplasia.
5th pattern is round cell with increased stromal cellularity composed of cells with round bland nuclei. In contrast, stromal BPH nuclei are oval and spindled. Can have associated complex glandular proliferations. Abrupt demarcation between normal stroma and STUMP.
The 2nd pattern of STUMP most closely resembles stromal nodular BPH. BPH stroma is not as cellular as 2nd pattern of STUMP and lacks eosinophilic cytoplasm. Also in contrast to STUMP, BPH stroma has nodularity and numerous thick walled small arterioles. These vessels are distinctive, as typically such small vessels would not have thick walls. This feature is particularly helpful on needle biopsy, where the multinodularity cannot be appreciated. This case further mimicked a neoplasm with the presence of necrosis. BPH, stromal or glandular-stromal can undergo infarction which correlates with size of BPH and also systemic atherosclerosis. The
current case lacks the marked hypercellularity, pleomorphism, and mitotic activity seen in stromal sarcomas. Stromal nodular BPH may be extensive without any prostate glands mimicking a stroma neoplasm.
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen
Clinical History. A 65 year old man underwent a TURB for a bladder mass.
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Clinical History. A 65 year old man underwent a TURB for a bladder mass.
Correct
Answer: B.
Histology: The tumor consists of a signet ring cell adenocarcinoma. Focally, there are well-formed glands with a few goblet cells and areas with loss of goblet cells and high grade dysplasia.
Discussion. If one had only the signet ring cell adenocarcinoma on a TURB, typically one would have to state in the pathology report, that this could be primary in the bladder but could not rule out spread from a GI primary. The only stain that could possibly help would be GATA3 which is positive in a minority of signet ring cell adenocarcinomas arising in the bladder. Other markers of intestinal differentiation, such as CDX2 or STAB2 in addition to CK7 or CK20 can be seen in both bladder and GI adenocarcinomas and are not helpful in this differential diagnosis. However, in this case there are other features that allow us to determine that this tumor arose in the bladder. The finding of a precursor lesion of glands with intestinal metaplasia with high grade dysplasia indicates a bladder primary. Bladder adenocarcinomas arise from two pathways. One is, as in this case, from intestinal metaplasia that develops dysplasia. The other is adenocarcinomas that arise from villous adenomas of the bladder. The glandular tumors and their precursors in the bladder are entirely analogous to their GI counterparts in their morphology and immunophenotype.
Incorrect
Answer: B.
Histology: The tumor consists of a signet ring cell adenocarcinoma. Focally, there are well-formed glands with a few goblet cells and areas with loss of goblet cells and high grade dysplasia.
Discussion. If one had only the signet ring cell adenocarcinoma on a TURB, typically one would have to state in the pathology report, that this could be primary in the bladder but could not rule out spread from a GI primary. The only stain that could possibly help would be GATA3 which is positive in a minority of signet ring cell adenocarcinomas arising in the bladder. Other markers of intestinal differentiation, such as CDX2 or STAB2 in addition to CK7 or CK20 can be seen in both bladder and GI adenocarcinomas and are not helpful in this differential diagnosis. However, in this case there are other features that allow us to determine that this tumor arose in the bladder. The finding of a precursor lesion of glands with intestinal metaplasia with high grade dysplasia indicates a bladder primary. Bladder adenocarcinomas arise from two pathways. One is, as in this case, from intestinal metaplasia that develops dysplasia. The other is adenocarcinomas that arise from villous adenomas of the bladder. The glandular tumors and their precursors in the bladder are entirely analogous to their GI counterparts in their morphology and immunophenotype.
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen
A 39 year old man presented with a 1 cm testicular mass. An orchiectomy was performed.
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A 39 year old man presented with a 1 cm testicular mass. An orchiectomy was performed.
Correct
Answer: B
Histological Description: The testis shows a collection of dilated glands variably lined by respiratory epithelium or goblet cells. The glands are surrounded by smooth muscle and the glands lack cytological atypia. The surround testis shows normal spermatogenesis.
Discussion: Prior to 2013, all teratomas in the testis occurring in postpubertal males were considered malignant, even if histologically they appeared benign. Subsequently, it has been recognized that there exists benign teratomas termed Prepubertal-type teratomas, which although typically seen in prepubertal males can rarely also be seen postpubertally. The new term for malignant teratomas is Postpubertal-type teratomas, which is only seen in postpubertal males. Most postpubertal-type teratomas have more solid areas, cytological atypia, or lack the organoid architecture resembling normal GI or respiratory structures. However on occasion can mimic prepubertal-type teratomas. In addition in the surrounding testis of postpubertal-type teratomas there is typically atrophic seminiferous tubules with Sertoli only pattern and also more focally germ cell neoplasia in situ. Coarse calcifications are also occasionally seen. In contrast, the testis surrounding prepubertal-type teratomas is normal. Prepubertal-type teratomas also lack amplification of isochromosome 12p by FISH, which is seen in the vast majority of postpubertal-type teratomas similar to other malignant germ cell tumors. In the current case, the tumor lacked amplification of i12p.
Reference:
Zhang C, Berney DM, Hirsch MS, Cheng L, Ulbright TM. Evidence supporting the existence of benign teratomas of the postpubertal testis: a clinical, histopathologic, and molecular genetic analysis of 25 cases. Am J Surg Pathol. 201337(6):827-35.
Incorrect
Answer: B
Histological Description: The testis shows a collection of dilated glands variably lined by respiratory epithelium or goblet cells. The glands are surrounded by smooth muscle and the glands lack cytological atypia. The surround testis shows normal spermatogenesis.
Discussion: Prior to 2013, all teratomas in the testis occurring in postpubertal males were considered malignant, even if histologically they appeared benign. Subsequently, it has been recognized that there exists benign teratomas termed Prepubertal-type teratomas, which although typically seen in prepubertal males can rarely also be seen postpubertally. The new term for malignant teratomas is Postpubertal-type teratomas, which is only seen in postpubertal males. Most postpubertal-type teratomas have more solid areas, cytological atypia, or lack the organoid architecture resembling normal GI or respiratory structures. However on occasion can mimic prepubertal-type teratomas. In addition in the surrounding testis of postpubertal-type teratomas there is typically atrophic seminiferous tubules with Sertoli only pattern and also more focally germ cell neoplasia in situ. Coarse calcifications are also occasionally seen. In contrast, the testis surrounding prepubertal-type teratomas is normal. Prepubertal-type teratomas also lack amplification of isochromosome 12p by FISH, which is seen in the vast majority of postpubertal-type teratomas similar to other malignant germ cell tumors. In the current case, the tumor lacked amplification of i12p.
Reference:
Zhang C, Berney DM, Hirsch MS, Cheng L, Ulbright TM. Evidence supporting the existence of benign teratomas of the postpubertal testis: a clinical, histopathologic, and molecular genetic analysis of 25 cases. Am J Surg Pathol. 201337(6):827-35.
Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen.
Adult female with kidney tumor.
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Adult female with kidney tumor.
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Correct: B
Histology: This solid and cystic tumor is composed of tumor cells with abundant eosinophilic cytoplasm, enlarged nuclei and prominent nucleoli. The cysts are lined by cells exhibiting hobnail arrangement with voluminous eosinophilic cytoplasm. The cells in the solid areas typically showed diffuse and compact acinar or nested growth. The cytoplasm shows a characteristic granular, fine or coarse stippling, consisting of basophilic to purple inclusions.
Discussion: Eosinophilic solid and cystic RCC (ESC RCC)shows the characteristic morphology of the case presented here, with often tan, solid, and cystic gross appearance and cells exhibiting eosinophilic, voluminous cytoplasm with granular stippling. The tumors are typically low stage and often show prominent nucleoli. There is frequent CK20 positivity, whereas CK7 is usually negative or only focally positive. Cathepsin-K can be positive, which is also positive in epithelioid AMLs but AMLs are negative for PAX8 while ESC RCC is most frequently positive. ESCs RCC are associated with mutations in the TSC1 gene, with or without association with tuberous sclerosis syndrome. While most patients present with organ confined disease and have a favorable outcome, rare cases of metastatic ESC RCC have been documented.
References:
1. Trpkov K, Hes O, Bonert M, et al. Eosinophilic, solid, and cystic renal cell carcinoma: clinicopathologic study of 16 unique, sporadic neoplasms occurring in women. Am J Surg Pathol. 2016;40:60–71.
2. Trpkov K, Abou-Ouf H, Hes O, Lopez JI, Nesi G, Comperat E, Sibony M, Osunkoya AO, Zhou M, Gokden N, Leroy X, Berney DM, Werneck Cunha I, Musto ML, Athanazio DA, Yilmaz A, Donnelly B, Hyndman E, Gill AJ, McKenney JK, Bismar TA. Eosinophilic Solid and Cystic Renal Cell Carcinoma (ESC RCC): Further Morphologic and Molecular Characterization of ESC RCC as a Distinct Entity. Am J Surg Pathol. 2017 Oct;41(10):1299-1308.
3. Palsgrove DN, Li Y, Pratilas CA, Lin MT, Pallavajjalla A, Gocke C, De Marzo AM, Matoso A, Netto GJ, Epstein JI, Argani P. Eosinophilic Solid and Cystic (ESC) Renal CellCarcinomas Harbor TSC Mutations: Molecular Analysis Supports an Expanding Clinicopathologic Spectrum. Am J Surg Pathol. 2018 Sep;42(9):1166-1181.
Incorrect
Correct: B
Histology: This solid and cystic tumor is composed of tumor cells with abundant eosinophilic cytoplasm, enlarged nuclei and prominent nucleoli. The cysts are lined by cells exhibiting hobnail arrangement with voluminous eosinophilic cytoplasm. The cells in the solid areas typically showed diffuse and compact acinar or nested growth. The cytoplasm shows a characteristic granular, fine or coarse stippling, consisting of basophilic to purple inclusions.
Discussion: Eosinophilic solid and cystic RCC (ESC RCC)shows the characteristic morphology of the case presented here, with often tan, solid, and cystic gross appearance and cells exhibiting eosinophilic, voluminous cytoplasm with granular stippling. The tumors are typically low stage and often show prominent nucleoli. There is frequent CK20 positivity, whereas CK7 is usually negative or only focally positive. Cathepsin-K can be positive, which is also positive in epithelioid AMLs but AMLs are negative for PAX8 while ESC RCC is most frequently positive. ESCs RCC are associated with mutations in the TSC1 gene, with or without association with tuberous sclerosis syndrome. While most patients present with organ confined disease and have a favorable outcome, rare cases of metastatic ESC RCC have been documented.
References:
1. Trpkov K, Hes O, Bonert M, et al. Eosinophilic, solid, and cystic renal cell carcinoma: clinicopathologic study of 16 unique, sporadic neoplasms occurring in women. Am J Surg Pathol. 2016;40:60–71.
2. Trpkov K, Abou-Ouf H, Hes O, Lopez JI, Nesi G, Comperat E, Sibony M, Osunkoya AO, Zhou M, Gokden N, Leroy X, Berney DM, Werneck Cunha I, Musto ML, Athanazio DA, Yilmaz A, Donnelly B, Hyndman E, Gill AJ, McKenney JK, Bismar TA. Eosinophilic Solid and Cystic Renal Cell Carcinoma (ESC RCC): Further Morphologic and Molecular Characterization of ESC RCC as a Distinct Entity. Am J Surg Pathol. 2017 Oct;41(10):1299-1308.
3. Palsgrove DN, Li Y, Pratilas CA, Lin MT, Pallavajjalla A, Gocke C, De Marzo AM, Matoso A, Netto GJ, Epstein JI, Argani P. Eosinophilic Solid and Cystic (ESC) Renal CellCarcinomas Harbor TSC Mutations: Molecular Analysis Supports an Expanding Clinicopathologic Spectrum. Am J Surg Pathol. 2018 Sep;42(9):1166-1181.
Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen.
Adult male with history of germ cell tumor of testis, now with lung tumor.
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Adult male with history of germ cell tumor of testis, now with lung tumor.
Correct
Correct: B
Histology: Sections show a solid mass composed of round cells with eosinophilic cytoplasm with single nuclei and peripheral multinucleated cells with more amphophilic cytoplasm.
Discussion: Choriocarcinoma frequently metastasizes to the lung and to many other organs causing bleeding as a clinical presentation, either in the form . In this case, the syncytiotrophoblastic cells are subtle and located towards the edge of the tumor, which could make its recognition more challenging. In contrast to seminoma and embryonal carcinoma, choriocarcinoma is negative for OCT3/4 and positive for hCG, SALL4, and GATA3. Cytokeratin stains can be positive leading to confusion with carcinoma. Serum beta-hCG is elevated.
References
1. Elzamly S, Torabi A, Padilla O. Testicular Choriocarcinoma Metastasizing to the Small Bowel Causing Intussusception: Case Report. J Gastrointest Cancer. 2019 Dec;50(4):1005-1008.
2. Lee SC, Kim KH, Kim SH, Lee NS, Park HS, Won JH. Mixed testicular germ cell tumor presenting as metastatic pure choriocarcinoma involving multiple lung metastases that was effectively treated with high-dose chemotherapy. Cancer Res Treat. 2009 Dec;41(4):229-32.
Incorrect
Correct: B
Histology: Sections show a solid mass composed of round cells with eosinophilic cytoplasm with single nuclei and peripheral multinucleated cells with more amphophilic cytoplasm.
Discussion: Choriocarcinoma frequently metastasizes to the lung and to many other organs causing bleeding as a clinical presentation, either in the form . In this case, the syncytiotrophoblastic cells are subtle and located towards the edge of the tumor, which could make its recognition more challenging. In contrast to seminoma and embryonal carcinoma, choriocarcinoma is negative for OCT3/4 and positive for hCG, SALL4, and GATA3. Cytokeratin stains can be positive leading to confusion with carcinoma. Serum beta-hCG is elevated.
References
1. Elzamly S, Torabi A, Padilla O. Testicular Choriocarcinoma Metastasizing to the Small Bowel Causing Intussusception: Case Report. J Gastrointest Cancer. 2019 Dec;50(4):1005-1008.
2. Lee SC, Kim KH, Kim SH, Lee NS, Park HS, Won JH. Mixed testicular germ cell tumor presenting as metastatic pure choriocarcinoma involving multiple lung metastases that was effectively treated with high-dose chemotherapy. Cancer Res Treat. 2009 Dec;41(4):229-32.
Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen
Adult male with a paratesticular mass
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Adult male with a paratesticular mass
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Correct: D
Histology: Sections show a proliferation of spindle cells with small nuclei and hyperchromatic chromatin and indistinct cell borders. These cells have an infiltrative growth and involve adipose tissue, extending in fibrous septae.
Discussion: Diffuse neurofibromas are more common in the head and neck region of children and young adults but can occur anywhere. A subset of them is associated with neurofibromatosis syndrome but can also occur sporadically. As its name implies, this neurofibroma is poorly defined and spreads extensively along connective tissue septae and between fat cells. It differs from conventional neurofibroma in that it has a uniform matrix of fine fibrillary collagen. Cellularity is usually low. The tumor shows occasional pseudomeissnerian body-like structures. Entrapped large vessels are characteristic. Extremely rarely, diffuse neurofibromas may progress to malignant peripheral nerve sheath tumor. By immunohistochemistry, the tumor is diffusely positive for S100, SOX10 and CD34 and negative for MUC4 and MDM2.
References:
Schaefer IM and Fletcher CD. Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases. Am J Surg Pathol. 2015;39(9):1234-1241.
Incorrect
Correct: D
Histology: Sections show a proliferation of spindle cells with small nuclei and hyperchromatic chromatin and indistinct cell borders. These cells have an infiltrative growth and involve adipose tissue, extending in fibrous septae.
Discussion: Diffuse neurofibromas are more common in the head and neck region of children and young adults but can occur anywhere. A subset of them is associated with neurofibromatosis syndrome but can also occur sporadically. As its name implies, this neurofibroma is poorly defined and spreads extensively along connective tissue septae and between fat cells. It differs from conventional neurofibroma in that it has a uniform matrix of fine fibrillary collagen. Cellularity is usually low. The tumor shows occasional pseudomeissnerian body-like structures. Entrapped large vessels are characteristic. Extremely rarely, diffuse neurofibromas may progress to malignant peripheral nerve sheath tumor. By immunohistochemistry, the tumor is diffusely positive for S100, SOX10 and CD34 and negative for MUC4 and MDM2.
References:
Schaefer IM and Fletcher CD. Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases. Am J Surg Pathol. 2015;39(9):1234-1241.
Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen.
This is a 45 year old male with a testicular mass.
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This is a 45 year old male with a testicular mass.
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Answer: B. Adenomatoid tumor
Histologic Description: This lesion is based in the tunica or capsule of the testis, not the seminiferous tubules though it does abut the latter. The lesional cells are cord like and cuboidal in their appearance, with frequent vacuoles that mimic signet ring cell morphology. There is no atypia or significant mitotic activity. The lesional cells are immunoreactive for calretinin and not for CEA, confirming their mesothelial derivation.
Differential Diagnosis: Metastatic signet ring cell carcinoma would have a more destructive growth pattern, would not label for mesothelial markers, and would contain intracellular mucin in most cases. Seminomas should be centered in the testes, and would typically have a solid pattern with lymphoid rich septa. Yolk sac tumors may have prominent vacuoles, but these would demonstrate more atypia and be centered within the testes. None of the entities in the differential diagnosis would label for the mesothelial marker calretinin.
Adenomatoid tumors are well known mimics of neoplasia around the testes that can be encountered on frozen section. Others include the oncocytic tumors of the andrenogenital syndrome that mimic Leydig cell tumor, testicular epidermoid cysts that can mimic a teratoma, and reactive mesothelial proliferations.
Incorrect
Answer: B. Adenomatoid tumor
Histologic Description: This lesion is based in the tunica or capsule of the testis, not the seminiferous tubules though it does abut the latter. The lesional cells are cord like and cuboidal in their appearance, with frequent vacuoles that mimic signet ring cell morphology. There is no atypia or significant mitotic activity. The lesional cells are immunoreactive for calretinin and not for CEA, confirming their mesothelial derivation.
Differential Diagnosis: Metastatic signet ring cell carcinoma would have a more destructive growth pattern, would not label for mesothelial markers, and would contain intracellular mucin in most cases. Seminomas should be centered in the testes, and would typically have a solid pattern with lymphoid rich septa. Yolk sac tumors may have prominent vacuoles, but these would demonstrate more atypia and be centered within the testes. None of the entities in the differential diagnosis would label for the mesothelial marker calretinin.
Adenomatoid tumors are well known mimics of neoplasia around the testes that can be encountered on frozen section. Others include the oncocytic tumors of the andrenogenital syndrome that mimic Leydig cell tumor, testicular epidermoid cysts that can mimic a teratoma, and reactive mesothelial proliferations.
Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen.
This is a 59 year old male who underwent a splenectomy and is found to have a mass lesion.
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This is a 59 year old male who underwent a splenectomy and is found to have a mass lesion.
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Answer: A. EBV positive follicular dendritic cell tumor
Histologic Description: This is a lymphoid predominant lesion, but the primary neoplastic cells are spindled cells that are closely associated with small lymphocytes. The neoplastic spindle cells have vesicular nuclei and open chromatin, which makes them easy to dismiss on H&E sections These cells are highlighted by in situ hybridization for EBER RNA transcripts. Within the lesion, occasional blood vessels show fibrinoid deposits and are ectatic.
Differential Diagnosis: Inflammatory myofibroblastic tumors typically are immunoreactive for ALK and demonstrate ALK gene rearrangements. The spindle cells in such lesions are typically more prominent. Low-grade lymphoma is suggested by the morphology, but can be excluded by demonstration of a normal pattern of immunoreactivity for B and T cell markers and they lack of immunoglobulin gene rearrangements. Occasionally, one can see ectopic thymoma in the abdomen, though presentation in the spleen would be almost unheard of. Thymomas would label for cytokeratin and have a prominent T lymphocyte background.
EBV positive follicular dendritic cell tumors of this morphology have been called inflammatory pseudotumor like. They typically but not always affect the spleen and liver of Asian adults. They are often immunoreactive for follicular dendritic cell markers CD21 and CD35, but not always. They are considered low-grade malignancies.
Reference: Cheuk et al. Am J Surg Pathol. 2001; 25:721-731.
Incorrect
Answer: A. EBV positive follicular dendritic cell tumor
Histologic Description: This is a lymphoid predominant lesion, but the primary neoplastic cells are spindled cells that are closely associated with small lymphocytes. The neoplastic spindle cells have vesicular nuclei and open chromatin, which makes them easy to dismiss on H&E sections These cells are highlighted by in situ hybridization for EBER RNA transcripts. Within the lesion, occasional blood vessels show fibrinoid deposits and are ectatic.
Differential Diagnosis: Inflammatory myofibroblastic tumors typically are immunoreactive for ALK and demonstrate ALK gene rearrangements. The spindle cells in such lesions are typically more prominent. Low-grade lymphoma is suggested by the morphology, but can be excluded by demonstration of a normal pattern of immunoreactivity for B and T cell markers and they lack of immunoglobulin gene rearrangements. Occasionally, one can see ectopic thymoma in the abdomen, though presentation in the spleen would be almost unheard of. Thymomas would label for cytokeratin and have a prominent T lymphocyte background.
EBV positive follicular dendritic cell tumors of this morphology have been called inflammatory pseudotumor like. They typically but not always affect the spleen and liver of Asian adults. They are often immunoreactive for follicular dendritic cell markers CD21 and CD35, but not always. They are considered low-grade malignancies.
Reference: Cheuk et al. Am J Surg Pathol. 2001; 25:721-731.
Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen.
This is an 84 year old female with an abdominal mass and a 35 year history of “recurrent sarcoma”.
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This is an 84 year old female with an abdominal mass and a 35 year history of “recurrent sarcoma”.
Correct
Answer: B. Low grade fibromyxoid sarcoma
Histologic Description: This is a bland spindle cell lesion composed of spindles fibroblastic-type cells in a variably collagenous and myxoid background. Blood vessels are arranged in thin walled arcades in the myxoid areas, but are sparse in the more collagenous areas. Mitotic activity is scant. The neoplastic cells are immunoreactive for MUC4 and focally for EMA, but not for S100 protein.
Differential Diagnosis: Myxofibrosarcoma is a more pleomorphic myxoid sarcoma which is typically superficially located on the extremities. It lacks the characteristic translocation of low grade fibromyxoid sarcoma. Myxoid liposarcoma would demonstrate uniform lipoblastic cells characterized by vacuoles with indent the nucleus, and a characteristic t(12;16) translocation resulting in a FUS-DDIT3 gene fusion. Neurofibromas feature bland spindle cells with collagen that has a “shredded-carrot” pattern, along with prominent mast cells. Neurofibroma should demonstrate some immunoreactivity for S100 protein.
Low grade fibromyxoid sarcomas are bland neoplasms that are frequently mistaken for benign processes such as perineurioma and neurofibroma. The fact that they can focally stain for EMA is a pitfall that could lead to a misdiagnosis of perineurioma. MUC4 immunoreactivity is very useful in this differential diagnosis. Low grade fibromyxoid sarcoma is typically characterized by a t(7;16) translocation resulting in a FUS-CREB3L2 gene fusion. Sclerosing epithelioid fibrosarcoma is thought to be the intermediate/higher grade form of low grade fibromyxoid sarcoma. Cases with a sclerosing epithelioid fibrosarcoma pattern more commonly demonstrate the EWSR1-CREB3L1 or CREB3L2 gene fusions.
Incorrect
Answer: B. Low grade fibromyxoid sarcoma
Histologic Description: This is a bland spindle cell lesion composed of spindles fibroblastic-type cells in a variably collagenous and myxoid background. Blood vessels are arranged in thin walled arcades in the myxoid areas, but are sparse in the more collagenous areas. Mitotic activity is scant. The neoplastic cells are immunoreactive for MUC4 and focally for EMA, but not for S100 protein.
Differential Diagnosis: Myxofibrosarcoma is a more pleomorphic myxoid sarcoma which is typically superficially located on the extremities. It lacks the characteristic translocation of low grade fibromyxoid sarcoma. Myxoid liposarcoma would demonstrate uniform lipoblastic cells characterized by vacuoles with indent the nucleus, and a characteristic t(12;16) translocation resulting in a FUS-DDIT3 gene fusion. Neurofibromas feature bland spindle cells with collagen that has a “shredded-carrot” pattern, along with prominent mast cells. Neurofibroma should demonstrate some immunoreactivity for S100 protein.
Low grade fibromyxoid sarcomas are bland neoplasms that are frequently mistaken for benign processes such as perineurioma and neurofibroma. The fact that they can focally stain for EMA is a pitfall that could lead to a misdiagnosis of perineurioma. MUC4 immunoreactivity is very useful in this differential diagnosis. Low grade fibromyxoid sarcoma is typically characterized by a t(7;16) translocation resulting in a FUS-CREB3L2 gene fusion. Sclerosing epithelioid fibrosarcoma is thought to be the intermediate/higher grade form of low grade fibromyxoid sarcoma. Cases with a sclerosing epithelioid fibrosarcoma pattern more commonly demonstrate the EWSR1-CREB3L1 or CREB3L2 gene fusions.
Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen.
A 65 year old man underwent a radical nephrectomy for a 6.2 cm mass.
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1. Question
A 65 year old man underwent a radical nephrectomy for a 6.2 cm mass.
Choose the correct diagnosis:
Correct
Answer: D
Histological Description: There is an admixture of compressed elongated tubular and focal spindle cells with light eosinophilic cytoplasm. The epithelial cells are bland with small ovoid-round nuclei and inconspicuous nucleoli. Mitotic activity is low. A variable amount of stromal bubbly eosinophilic mucin is present.
Discussion: The histological findings in this case are typical of a mucinous tubular spindle cell carcinoma. The proportions of the three elements can vary, such that some of these tumors may have only focal spindle cell or mucinous features. By definition, these are histologically low grade tumors. There are some tumors that resemble mucinous tubular spindle cell carcinoma yet have overt cytological atypia. There is some data that suggests that these higher grade lesions share molecular features with mucinous tubular spindle cell carcinoma. However, in order to keep “mucinous tubular spindle cell carcinoma” for an indolent neoplasm, higher grade similar lesions are designated as renal cell carcinoma with features of mucinous tubular spindle cell carcinoma yet with too much cytological atypia for that diagnosis. The prognosis of mucinous tubular spindle cell carcinoma is excellent with only rare cases with metastases.
Incorrect
Answer: D
Histological Description: There is an admixture of compressed elongated tubular and focal spindle cells with light eosinophilic cytoplasm. The epithelial cells are bland with small ovoid-round nuclei and inconspicuous nucleoli. Mitotic activity is low. A variable amount of stromal bubbly eosinophilic mucin is present.
Discussion: The histological findings in this case are typical of a mucinous tubular spindle cell carcinoma. The proportions of the three elements can vary, such that some of these tumors may have only focal spindle cell or mucinous features. By definition, these are histologically low grade tumors. There are some tumors that resemble mucinous tubular spindle cell carcinoma yet have overt cytological atypia. There is some data that suggests that these higher grade lesions share molecular features with mucinous tubular spindle cell carcinoma. However, in order to keep “mucinous tubular spindle cell carcinoma” for an indolent neoplasm, higher grade similar lesions are designated as renal cell carcinoma with features of mucinous tubular spindle cell carcinoma yet with too much cytological atypia for that diagnosis. The prognosis of mucinous tubular spindle cell carcinoma is excellent with only rare cases with metastases.
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