Presented by Dr. Pedram Argani and prepared by Dr. Maryam Shabihkhani
87 year old female with a 1cm breast mass
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87 year old female with a 1cm breast mass
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Histologic Description: This is a fairly well delineated fibroblastic nodule with extensive hyalinization. There is subtle infiltration at the edge of the lesion, and abrupt variations in cellularity, which raise suspicion for metaplastic carcinoma. The spindle cells are diffusely immunoreactive for high molecular weight cytokeratin (34BE12), supporting the diagnosis of spindle cell metaplastic carcinoma.
Differential Diagnosis: Fibroadenoma would not label for high molecular weight cytokeratin, and typically labels for CD34. Sclerosed papillomas often have a hyalinized rounded appearance, but would not demonstrate high molecular weight cytokeratin labeling. Duct ectasia is characterized by markedly dilated ducts associated with squamous metaplasia and periductal chronic inflammation and histiocytes, not seen in the current case.
This case illustrates a subtle low grade spindle cell metaplastic carcinoma. Clues to the diagnosis include abrupt transitions in cellularity within the lesion, and the layered, plaque like hyaline fibrosis within the lesion.
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Correct: C
Histologic Description: This is a fairly well delineated fibroblastic nodule with extensive hyalinization. There is subtle infiltration at the edge of the lesion, and abrupt variations in cellularity, which raise suspicion for metaplastic carcinoma. The spindle cells are diffusely immunoreactive for high molecular weight cytokeratin (34BE12), supporting the diagnosis of spindle cell metaplastic carcinoma.
Differential Diagnosis: Fibroadenoma would not label for high molecular weight cytokeratin, and typically labels for CD34. Sclerosed papillomas often have a hyalinized rounded appearance, but would not demonstrate high molecular weight cytokeratin labeling. Duct ectasia is characterized by markedly dilated ducts associated with squamous metaplasia and periductal chronic inflammation and histiocytes, not seen in the current case.
This case illustrates a subtle low grade spindle cell metaplastic carcinoma. Clues to the diagnosis include abrupt transitions in cellularity within the lesion, and the layered, plaque like hyaline fibrosis within the lesion.
Presented by Dr. Pedram Argani and prepared by Dr. Maryam Shabihkhani
32 year old male with a paraspinal mass
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32 year old male with a paraspinal mass
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Histologic Description: This is a highly cellular spindle cell neoplasm, with occasional mitotic figures. The neoplasm permeates bone, which suggests an aggressive process. The neoplasm is diffusely immunoreactive for S100 protein, and other areas demonstrates the hyalinized vessels and lower cellularity typical of schwannoma.
Differential Diagnosis: Rhabdomyosarcoma would label for myogenin and desmin, it would have more eosinophilic cytoplasm. Leiomyosarcoma would demonstrate more eosinophilic cytoplasm and label for desmin. Malignant peripheral nerve sheath tumors are typically not diffusely S100 positive like schwannoma, and demonstrate greater atypia and mitotic activity.
Cellular schwannomas may erode bone and demonstrate mitotic activity, mimicking a malignancy.
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Correct: D
Histologic Description: This is a highly cellular spindle cell neoplasm, with occasional mitotic figures. The neoplasm permeates bone, which suggests an aggressive process. The neoplasm is diffusely immunoreactive for S100 protein, and other areas demonstrates the hyalinized vessels and lower cellularity typical of schwannoma.
Differential Diagnosis: Rhabdomyosarcoma would label for myogenin and desmin, it would have more eosinophilic cytoplasm. Leiomyosarcoma would demonstrate more eosinophilic cytoplasm and label for desmin. Malignant peripheral nerve sheath tumors are typically not diffusely S100 positive like schwannoma, and demonstrate greater atypia and mitotic activity.
Cellular schwannomas may erode bone and demonstrate mitotic activity, mimicking a malignancy.
Presented by Dr. Pedram Argani and prepared by Dr. Maryam Shabihkhani
18 year old male with a breast mass
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18 year old male with a breast mass
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Correct: B
Histologic Description: This is an infiltrative ductal process associated with an irregular border and mild stromal desmoplasia. The cells form tubules and cribriform structures and are associated with intracellular and extracellular eosinophilic secretions. The neoplasm demonstrates minimal immunoreactivity for estrogen receptor and progesterone receptor, unlike most low nuclear grade breast cancers. FISH demonstrates rearrangement of the ETV6 gene, supporting the diagnosis of invasive secretory carcinoma.
Differential Diagnosis: Conventional invasive ductal carcinoma would be distinctly unusual in this age group and gender, and does not harbor ETV6 gene rearrangements. Florid papillomatosis of the nipple would have an overall circumscribed appearance, and have bilayered epithelium containing both myoepithelial and ductal cells. Gynecomastia would feature prominent periductal edema and fibrosis, associated with micropapillary duct hyperplasia.
Invasive secretory carcinoma is a special favorable type of breast cancer associated with the ETV6-NTRK3 gene fusion. The latter is also seen in a variety of other neoplasms, including infantile fibrosarcoma, cellular congenital mesoblastic nephroma, and mammary analog secretory carcinoma of salivary glands. The latter is histologically identical to secretory carcinoma of the breast.
Incorrect
Correct: B
Histologic Description: This is an infiltrative ductal process associated with an irregular border and mild stromal desmoplasia. The cells form tubules and cribriform structures and are associated with intracellular and extracellular eosinophilic secretions. The neoplasm demonstrates minimal immunoreactivity for estrogen receptor and progesterone receptor, unlike most low nuclear grade breast cancers. FISH demonstrates rearrangement of the ETV6 gene, supporting the diagnosis of invasive secretory carcinoma.
Differential Diagnosis: Conventional invasive ductal carcinoma would be distinctly unusual in this age group and gender, and does not harbor ETV6 gene rearrangements. Florid papillomatosis of the nipple would have an overall circumscribed appearance, and have bilayered epithelium containing both myoepithelial and ductal cells. Gynecomastia would feature prominent periductal edema and fibrosis, associated with micropapillary duct hyperplasia.
Invasive secretory carcinoma is a special favorable type of breast cancer associated with the ETV6-NTRK3 gene fusion. The latter is also seen in a variety of other neoplasms, including infantile fibrosarcoma, cellular congenital mesoblastic nephroma, and mammary analog secretory carcinoma of salivary glands. The latter is histologically identical to secretory carcinoma of the breast.
Presented by Dr. Cimino-Mathews and prepared by Dr. Maryam Shabihkhani
50 year-old male with a spine mass
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50 year-old male with a spine mass
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Correct: Schwannoma
Histology: The lesion is a large multi-lobated mass comprised of spindle cells with wavy nuclei and tapering ends. In most areas, the lesion is hypocellular with a myxoid matrix (Antoni B); some areas display more cellular areas (Antoni A). There are prominent bands of nuclear palisading (Verocay bodies). There are scattered blood vessels within the lesion, but there is no notable hyalinization. Mitotic activity is rare, and necrosis is absent. By immunohistochemistry, the lesion is diffusely positive for S-100.
Discussion: Schwannomas are benign peripheral nerve sheath tumors that are comprised of neoplastic Schwann cells. They can occur throughout the body, including in the spinal cord. The lesional cells characteristically display wavy nuclei with tapered ends that may be arranged in palisaded rows termed “Verocay bodies”. The stroma may be myxoid, as is seen in this case. Regions of hypercellularity are termed “Antoni A” regions, whereas hypocellular (and often myxoid) areas are terms “Antoni B” regions. There are a number of variant morphologies, including the cellular, epithelioid, and melanotic (pigmented) schwannoma variants. Marked degenerative atypia can be seen in “ancient” schwannomas. Schwannomas are diffusely immunoreactive for S-100 and Sox10; in contrast, neurofibromas are typically focally immunoreactive for S-100 and Sox10. The differential diagnosis of a spindle cell or myxoid lesion in the spinal cord includes chordoma, meningioma, myxopapillary ependymoma. In addition to histologic clues, immunohistochemistry can resolve the diadnosis. Chordomas are immunoreactive for brachyury in addition to S-100; meningiomas are immunoreactive for EMA and PR; and myxopapillary ependymomas are immunoreactive for GFAP, but can also sometimes label for S-100.
References
1.Jessen KR, Mirsky R. The origin and development of glial cells in peripheral nerves. Nat Rev Neurosci 2005;6(9):671-682.
2.Fisher C. Immunohistochemistry in diagnosis of soft tissue tumours. Histopathology 2011;58(7):1001-1012.
Incorrect
Correct: Schwannoma
Histology: The lesion is a large multi-lobated mass comprised of spindle cells with wavy nuclei and tapering ends. In most areas, the lesion is hypocellular with a myxoid matrix (Antoni B); some areas display more cellular areas (Antoni A). There are prominent bands of nuclear palisading (Verocay bodies). There are scattered blood vessels within the lesion, but there is no notable hyalinization. Mitotic activity is rare, and necrosis is absent. By immunohistochemistry, the lesion is diffusely positive for S-100.
Discussion: Schwannomas are benign peripheral nerve sheath tumors that are comprised of neoplastic Schwann cells. They can occur throughout the body, including in the spinal cord. The lesional cells characteristically display wavy nuclei with tapered ends that may be arranged in palisaded rows termed “Verocay bodies”. The stroma may be myxoid, as is seen in this case. Regions of hypercellularity are termed “Antoni A” regions, whereas hypocellular (and often myxoid) areas are terms “Antoni B” regions. There are a number of variant morphologies, including the cellular, epithelioid, and melanotic (pigmented) schwannoma variants. Marked degenerative atypia can be seen in “ancient” schwannomas. Schwannomas are diffusely immunoreactive for S-100 and Sox10; in contrast, neurofibromas are typically focally immunoreactive for S-100 and Sox10. The differential diagnosis of a spindle cell or myxoid lesion in the spinal cord includes chordoma, meningioma, myxopapillary ependymoma. In addition to histologic clues, immunohistochemistry can resolve the diadnosis. Chordomas are immunoreactive for brachyury in addition to S-100; meningiomas are immunoreactive for EMA and PR; and myxopapillary ependymomas are immunoreactive for GFAP, but can also sometimes label for S-100.
References
1.Jessen KR, Mirsky R. The origin and development of glial cells in peripheral nerves. Nat Rev Neurosci 2005;6(9):671-682.
2.Fisher C. Immunohistochemistry in diagnosis of soft tissue tumours. Histopathology 2011;58(7):1001-1012.
Presented by Dr. Cimino-Mathews and prepared by Dr. Maryam Shabihkhani
30 year-old female with a colon mass
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30 year-old female with a colon mass
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Correct: Endometriosis
Histology: This resection specimen contains a section of small bowel notable for irregularly arranged glands involving the serosal surface and muscularis propria of the bowel wall. The individual glands are lined by low cuboidal to columnar cells with uniform nuclei and no atypia. Many of the glands contain pale pink intraluminal secretions. Some glands have associated periglandular stroma comprised of bland spindled cells with associated chronic inflammatory cells, including hemosiderin-laden macrophages. There are associated extravasated red blood cells within this periglandular stroma. There is no surface dysplasia of the bowel mucosa.
Discussion: This patient’s obstructing colon mass is due to florid endometriosis resulting in large and small bowel adhesions and strictures. The individual glands are comprised of bland, cuboidal to columnar cells, which can be occasionally ciliated. The cells can variably appear proliferative, secretory or hyperplastic in response to the hormonal stimulus of the patient’s menstrual cycle. The glands are characteristically surrounded by endometrial-type stroma, consisting of small, blue wavy cells that may show hemosiderin deposition and hemosiderin-laden macrophages due to bleeding. The differential diagnosis and diagnostic pitfall is invasive adenocarcinoma, whether of colonic or endometrial origin. In cases of florid endometriosis, it is important to evaluate for the presence of complex or atypical hyperplasia; rarely, clear cell carcinoma can arise within endometriosis particularly endometriomas.
References
1.Bulun SE. Endometriosis. N Engl J Med 2009; 360:268-279
2.Clement PB. The pathology of endometriosis: a survey of the many faces of a common disease emphasizing diagnostic pitfalls and unusual and newly appreciated aspects. Adv Anat Pathol. 2007 Jul;14(4):241-60.
Incorrect
Correct: Endometriosis
Histology: This resection specimen contains a section of small bowel notable for irregularly arranged glands involving the serosal surface and muscularis propria of the bowel wall. The individual glands are lined by low cuboidal to columnar cells with uniform nuclei and no atypia. Many of the glands contain pale pink intraluminal secretions. Some glands have associated periglandular stroma comprised of bland spindled cells with associated chronic inflammatory cells, including hemosiderin-laden macrophages. There are associated extravasated red blood cells within this periglandular stroma. There is no surface dysplasia of the bowel mucosa.
Discussion: This patient’s obstructing colon mass is due to florid endometriosis resulting in large and small bowel adhesions and strictures. The individual glands are comprised of bland, cuboidal to columnar cells, which can be occasionally ciliated. The cells can variably appear proliferative, secretory or hyperplastic in response to the hormonal stimulus of the patient’s menstrual cycle. The glands are characteristically surrounded by endometrial-type stroma, consisting of small, blue wavy cells that may show hemosiderin deposition and hemosiderin-laden macrophages due to bleeding. The differential diagnosis and diagnostic pitfall is invasive adenocarcinoma, whether of colonic or endometrial origin. In cases of florid endometriosis, it is important to evaluate for the presence of complex or atypical hyperplasia; rarely, clear cell carcinoma can arise within endometriosis particularly endometriomas.
References
1.Bulun SE. Endometriosis. N Engl J Med 2009; 360:268-279
2.Clement PB. The pathology of endometriosis: a survey of the many faces of a common disease emphasizing diagnostic pitfalls and unusual and newly appreciated aspects. Adv Anat Pathol. 2007 Jul;14(4):241-60.
Presented by Dr. Cimino-Mathews and prepared by Dr. Maryam Shabihkhani
50 year-old male with a colon mass
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50 year-old male with a colon mass
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Correct: Inflammatory fibroid polyp
Histology: The large polypoid lesion seen on this histologic section was grossly attached to the cecal wall by a thin stalk. The surface of the lesion is lined by bland-appearing colonic mucosal glands with regions of acute inflammation and ulceration. The center of the polypoid lesion contains irregular but benign-appearing colonic glands; abundant goblet cells are present, and the cells have low nuclear-to-cytoplasmic ratios (i.e., there is no dysplasia). The stroma located in between the disorganized colonic glands contains bland spindled cells with blunt-ended euchromatic nuclei, occasional punctate nucleoli, and no atypia. There are numerous eosinophils within the stromal space, as well as prominent thick-walled blood vessels. There is a vague suggestion of “onion skinning” around the blood vessels, that is, perivascular edema with zones of spindled cells resembling onion peels.
Discussion: Inflammatory fibroid polyps are mesenchymal lesions of the gastrointestinal tract that are centered within the submucosa. Although traditionally thought to be most common in the stomach and small intestine, they can also occur in the large intestine as is seen in this case. The classic histolofic features are the presence of bland spindled cells, thick walled vessels with perivascular “onion skinning,” and prominent eosinophils. Inflammatory fibroid polyps can contain varying degrees of spindled cells within the stroma such that the differential diagnosis can vary widely depending upon the degree of cellularity. The differential diagnosis of lesions with prominent spindle cells includes gastrointestinal stromal tumor (GIST), fibromatosis, and schwannoma. Notably, inflammatory fibroid polyps are immunoreactive for CD34, as are GISTs; however, in contrast to GISTS, inflammatory fibroid polyps are negative for CKIT/CD117 and DOG1. Inflammatory fibroid polyps can become secondarily inflamed and ulcerated due to traumatic injury within the luminal gastrointestinal tract; when large, they can cause bowel obstruction. In contrast, inflammatory polyps (not inflammatory fibroid polyps) are pseudopolyps that occur in the setting of diffuse inflammation due to inflammatory bowel disease.
References:
1.Daum O, Hes O, Vanecek T, et al. Vanek’s tumor (inflammatory fibroid polyp). Report of 18 cases and comparison with three cases of original Vanek’s series. Ann Diagn Pathol. 2003;7:337-47.
2.Greenson JK. Gastrointestinal stromal tumors and other mesenchymal lesions of the gut. Mod Pathol. 2003;16:366-75.
3.Liu TC, Lin MT, Montgomery EA, Singhi AD. Inflammatory fibroid polyps of the gastrointestinal tract: spectrum of clinical, morphologic, and immunohistochemistry features. Am J Surg Pathol. 2013 Apr;37(4):586-92.
Incorrect
Correct: Inflammatory fibroid polyp
Histology: The large polypoid lesion seen on this histologic section was grossly attached to the cecal wall by a thin stalk. The surface of the lesion is lined by bland-appearing colonic mucosal glands with regions of acute inflammation and ulceration. The center of the polypoid lesion contains irregular but benign-appearing colonic glands; abundant goblet cells are present, and the cells have low nuclear-to-cytoplasmic ratios (i.e., there is no dysplasia). The stroma located in between the disorganized colonic glands contains bland spindled cells with blunt-ended euchromatic nuclei, occasional punctate nucleoli, and no atypia. There are numerous eosinophils within the stromal space, as well as prominent thick-walled blood vessels. There is a vague suggestion of “onion skinning” around the blood vessels, that is, perivascular edema with zones of spindled cells resembling onion peels.
Discussion: Inflammatory fibroid polyps are mesenchymal lesions of the gastrointestinal tract that are centered within the submucosa. Although traditionally thought to be most common in the stomach and small intestine, they can also occur in the large intestine as is seen in this case. The classic histolofic features are the presence of bland spindled cells, thick walled vessels with perivascular “onion skinning,” and prominent eosinophils. Inflammatory fibroid polyps can contain varying degrees of spindled cells within the stroma such that the differential diagnosis can vary widely depending upon the degree of cellularity. The differential diagnosis of lesions with prominent spindle cells includes gastrointestinal stromal tumor (GIST), fibromatosis, and schwannoma. Notably, inflammatory fibroid polyps are immunoreactive for CD34, as are GISTs; however, in contrast to GISTS, inflammatory fibroid polyps are negative for CKIT/CD117 and DOG1. Inflammatory fibroid polyps can become secondarily inflamed and ulcerated due to traumatic injury within the luminal gastrointestinal tract; when large, they can cause bowel obstruction. In contrast, inflammatory polyps (not inflammatory fibroid polyps) are pseudopolyps that occur in the setting of diffuse inflammation due to inflammatory bowel disease.
References:
1.Daum O, Hes O, Vanecek T, et al. Vanek’s tumor (inflammatory fibroid polyp). Report of 18 cases and comparison with three cases of original Vanek’s series. Ann Diagn Pathol. 2003;7:337-47.
2.Greenson JK. Gastrointestinal stromal tumors and other mesenchymal lesions of the gut. Mod Pathol. 2003;16:366-75.
3.Liu TC, Lin MT, Montgomery EA, Singhi AD. Inflammatory fibroid polyps of the gastrointestinal tract: spectrum of clinical, morphologic, and immunohistochemistry features. Am J Surg Pathol. 2013 Apr;37(4):586-92.
Presented by Dr. Andres Matoso and prepared by Dr. Maryam Shabihkhani
19 year old male with retroperitoneal mass
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19 year old male with retroperitoneal mass
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Correct: A
Histology: This tumor is composed of malignant teratoma and adjacent prominent blood vessel neoplasm with endothelial cells with marked nuclear atypia.
Discussion: This is an example of somatic malignancy (angiosarcoma) arising from a teratoma. The definition of somatic malignancy arising in teratoma is a teratoma with a secondary component that resembles a somatic-type malignant neoplasm as seen in other organs and tissues (e.g. sarcomas and carcinomas). The somatic type malignancy should have an infiltrative pattern and occupy at least a 4x field (5 mm). Sarcomas are the most common histologic type, and more than half of these are rhabdomyosarcomas. PNET/Ewing sarcomas are also relatively frequent but they do not harbor the typical ESWR1 translocation. Nephroblastomas (Wilms tumors) are rare but well documented.
References:
1. Comiter CV, Kibel AS, Richie JP, et al. Prognostic features of teratomas with malignant transformation: a clinicopathological study of 21 cases. J Urol. 1998;159:859-863.
2. Guo CC, Punar M, Contreras AL, et al. Testicular germ cell tumors with sarcomatous components: an analysis of 33 cases. Am J Surg Pathol. 2009;33:1173-1178.
3. Colecchia M, Necchi A, Paolini B, et al. Teratoma with somatic-type malignant components in germ cell tumors of the testis: a clinicopathologic analysis of 40 cases with outcome correlation. Int J Surg Pathol. 2011;19:321-327.
4. Ahmed T, Bosl GJ, Hajdu SI. Teratoma with malignant transformation in germ cell tumors in men. Cancer. 1985;56:860-863
Incorrect
Correct: A
Histology: This tumor is composed of malignant teratoma and adjacent prominent blood vessel neoplasm with endothelial cells with marked nuclear atypia.
Discussion: This is an example of somatic malignancy (angiosarcoma) arising from a teratoma. The definition of somatic malignancy arising in teratoma is a teratoma with a secondary component that resembles a somatic-type malignant neoplasm as seen in other organs and tissues (e.g. sarcomas and carcinomas). The somatic type malignancy should have an infiltrative pattern and occupy at least a 4x field (5 mm). Sarcomas are the most common histologic type, and more than half of these are rhabdomyosarcomas. PNET/Ewing sarcomas are also relatively frequent but they do not harbor the typical ESWR1 translocation. Nephroblastomas (Wilms tumors) are rare but well documented.
References:
1. Comiter CV, Kibel AS, Richie JP, et al. Prognostic features of teratomas with malignant transformation: a clinicopathological study of 21 cases. J Urol. 1998;159:859-863.
2. Guo CC, Punar M, Contreras AL, et al. Testicular germ cell tumors with sarcomatous components: an analysis of 33 cases. Am J Surg Pathol. 2009;33:1173-1178.
3. Colecchia M, Necchi A, Paolini B, et al. Teratoma with somatic-type malignant components in germ cell tumors of the testis: a clinicopathologic analysis of 40 cases with outcome correlation. Int J Surg Pathol. 2011;19:321-327.
4. Ahmed T, Bosl GJ, Hajdu SI. Teratoma with malignant transformation in germ cell tumors in men. Cancer. 1985;56:860-863
Presented by Dr. Andres Matoso and prepared by Dr. Maryam Shabihkhani
7 year old male with testicular pain
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7 year old male with testicular pain
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Correct: C
Histology. The testis shows diffuse hemorrhagic necrosis involving the entire parenchyma.
Discussion: Torsion of the testis occurs as a result of the testis rotating on its axis and compressing the blood supply of from the spermatic cord. The clinical presentation is of acute scrotal pain. In children, the main symptom could be abdominal pain (referred pain to the abdomen), which could lead to a delay in the diagnosis due to lack of examination of the genitalia. If attempts to reverse the torsion are unsuccessful, the management is orchiectomy. Histologically, the main differential is with vasculitis, in which specific vessels are affected by inflammation and a segment of the testicular parenchyma will be affected. In contrast to torsion, in vasculitis there is a sharp demarcation between the area of necrosis and vital parenchyma. In the initial phases of torsion, the parenchyma is diffusely hemorrhagic and undergoing necrosis, some of the seminiferous tubules may still appear vital. After 24 hours, the picture is of diffuse necrosis and the predominant picture is of ghost cells with the shape of seminiferous tubules (coagulative necrosis). Acute inflammation can be seen as a response to ischemia and should not be considered infectious orchitis.
References:
Differential diagnoses in Surgical Pathology. Johnathan Epstein and George Netto. Chapter 4. Testis.
Incorrect
Correct: C
Histology. The testis shows diffuse hemorrhagic necrosis involving the entire parenchyma.
Discussion: Torsion of the testis occurs as a result of the testis rotating on its axis and compressing the blood supply of from the spermatic cord. The clinical presentation is of acute scrotal pain. In children, the main symptom could be abdominal pain (referred pain to the abdomen), which could lead to a delay in the diagnosis due to lack of examination of the genitalia. If attempts to reverse the torsion are unsuccessful, the management is orchiectomy. Histologically, the main differential is with vasculitis, in which specific vessels are affected by inflammation and a segment of the testicular parenchyma will be affected. In contrast to torsion, in vasculitis there is a sharp demarcation between the area of necrosis and vital parenchyma. In the initial phases of torsion, the parenchyma is diffusely hemorrhagic and undergoing necrosis, some of the seminiferous tubules may still appear vital. After 24 hours, the picture is of diffuse necrosis and the predominant picture is of ghost cells with the shape of seminiferous tubules (coagulative necrosis). Acute inflammation can be seen as a response to ischemia and should not be considered infectious orchitis.
References:
Differential diagnoses in Surgical Pathology. Johnathan Epstein and George Netto. Chapter 4. Testis.
Presented by Dr. Andres Matoso and prepared by Dr. Maryam Shabihkhani
Adult male with testicular mass
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Adult male with testicular mass
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Correct: B
Histology: Well-circumscribed tumor composed of tubules and nests of tumor cells with small or inconspicuous nucleoli and clear cytoplasm in a hyalinized stroma.
Discussion: This is the classic morphology of a benign Sertoli cell tumor. Sertoli cell tumors are infrequent and constitute less than 1% of all testicular tumors. The most frequent clinical presentation is a painless testicular mass. Occasionally, patients will present with gynecomastia. The majority are sporadic but some are associated with androgen insensitivity syndrome, Carney syndrome or Peutz-Jeghers syndrome. Cord-like arrangements of the cells in scanty pauci-cellular stroma could mimic a neuroendocrine tumor, especially because a subset of Sertoli cell tumors are positive for neuroendocrine markers. Inhibin is positive but less frequently than in Leydig cell tumors. Other positive markers include EMA and SF1. Germ cell markers including PLAP, OCT3/4 and CD30 are negative. Size over 5 cm, cellular atypia, increased mitotic activity, necrosis, infiltrative pattern, extratesticular extension and vascular invasion are associated with malignancy.
Histology: Well-circumscribed tumor composed of tubules and nests of tumor cells with small or inconspicuous nucleoli and clear cytoplasm in a hyalinized stroma.
Discussion: This is the classic morphology of a benign Sertoli cell tumor. Sertoli cell tumors are infrequent and constitute less than 1% of all testicular tumors. The most frequent clinical presentation is a painless testicular mass. Occasionally, patients will present with gynecomastia. The majority are sporadic but some are associated with androgen insensitivity syndrome, Carney syndrome or Peutz-Jeghers syndrome. Cord-like arrangements of the cells in scanty pauci-cellular stroma could mimic a neuroendocrine tumor, especially because a subset of Sertoli cell tumors are positive for neuroendocrine markers. Inhibin is positive but less frequently than in Leydig cell tumors. Other positive markers include EMA and SF1. Germ cell markers including PLAP, OCT3/4 and CD30 are negative. Size over 5 cm, cellular atypia, increased mitotic activity, necrosis, infiltrative pattern, extratesticular extension and vascular invasion are associated with malignancy.
Presented by Dr. White and prepared by Austin McCuiston.
75 year old female with a lateral neck mass.
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75 year old female with a lateral neck mass.
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Answer: B
Histology: At low magnification, there is a cystic epithelial lined lesion with associated lymphoid tissue involving fibrovascular and adipose tissue. A small focus of benign salivary gland tissue is present. The epithelial lining shows an undulating interface with the associated lymphoid and fibrous tissue. On higher magnification, the epithelial cells show squamous features, with distinct cell borders, areas of keratinization, and prominent glyocgenated cytoplasm. The nuclei are hyperchromatic with varying degrees of atypia, and some showing marked pleomorphism. Scattered mitotic figures are identified, in addition to areas of necrosis. The associated lymphoid tissue shows scattered germinal centers, and areas with intact lymphoid sinuses are seen.
Discussion: Patient demographics and specific anatomic location are critical when assessing cystic epithelial lined lesions in the neck. Although the differential for cystic squamous lesions in the lateral neck includes branchial cleft cysts, a retrospective review by Gourin demonstrated that the incidence of metastatic squamous cell carcinoma in isolated lateral cervical cysts is significantly higher in patients greater than 40 years of age. Although the patient in this case had a known history of a primary head and neck SCC, it is not uncommon for a patient to present with a lateral neck SCC metastasis and an occult primary. Specifically this is well-described for HPV-related oropharyngeal SCC. In the absence of a known primary and a lateral neck SCC metastasis, it is prudent to perform p16 and confirmatory high risk HPV ISH. P16 positivity (> or equal to 70% nuclear and cytoplasmic expression with at least moderate to strong intensity) alone is not entirely specific for HPV related OPSCC, as p16 has been reported to be positive in up to 43% of benign lymphoepithelial cysts of the lateral neck (ie branchial cleft cyst) and parotid gland.
References:
Gourin CG, Johnson JT. Incidence of unsuspected metastases in lateral cervical cysts. Laryngoscope. 2000 Oct;110(10 Pt 1):1637-41. PubMed PMID: 11037817.
Cao D, Begum S, Ali SZ, Westra WH. Expression of p16 in benign and malignant cystic squamous lesions of the neck. Hum Pathol. 2010 Apr;41(4):535-9. doi: 10.1016/j.humpath.2009.09.006. Epub 2009 Dec 11. PubMed PMID: 20004950.
Fakhry C, Lacchetti C, Rooper LM, Jordan RC, Rischin D, Sturgis EM, Bell D, Lingen MW, Harichand-Herdt S, Thibo J, Zevallos J, Perez-Ordonez B. Human Papillomavirus Testing in Head and Neck Carcinomas: ASCO Clinical Practice Guideline Endorsement of the College of American Pathologists Guideline. J Clin Oncol. 2018 Sep 6:JCO1800684. doi: 10.1200/JCO.18.00684. [Epub ahead of print] PubMed PMID: 30188786.
Incorrect
Answer: B
Histology: At low magnification, there is a cystic epithelial lined lesion with associated lymphoid tissue involving fibrovascular and adipose tissue. A small focus of benign salivary gland tissue is present. The epithelial lining shows an undulating interface with the associated lymphoid and fibrous tissue. On higher magnification, the epithelial cells show squamous features, with distinct cell borders, areas of keratinization, and prominent glyocgenated cytoplasm. The nuclei are hyperchromatic with varying degrees of atypia, and some showing marked pleomorphism. Scattered mitotic figures are identified, in addition to areas of necrosis. The associated lymphoid tissue shows scattered germinal centers, and areas with intact lymphoid sinuses are seen.
Discussion: Patient demographics and specific anatomic location are critical when assessing cystic epithelial lined lesions in the neck. Although the differential for cystic squamous lesions in the lateral neck includes branchial cleft cysts, a retrospective review by Gourin demonstrated that the incidence of metastatic squamous cell carcinoma in isolated lateral cervical cysts is significantly higher in patients greater than 40 years of age. Although the patient in this case had a known history of a primary head and neck SCC, it is not uncommon for a patient to present with a lateral neck SCC metastasis and an occult primary. Specifically this is well-described for HPV-related oropharyngeal SCC. In the absence of a known primary and a lateral neck SCC metastasis, it is prudent to perform p16 and confirmatory high risk HPV ISH. P16 positivity (> or equal to 70% nuclear and cytoplasmic expression with at least moderate to strong intensity) alone is not entirely specific for HPV related OPSCC, as p16 has been reported to be positive in up to 43% of benign lymphoepithelial cysts of the lateral neck (ie branchial cleft cyst) and parotid gland.
References:
Gourin CG, Johnson JT. Incidence of unsuspected metastases in lateral cervical cysts. Laryngoscope. 2000 Oct;110(10 Pt 1):1637-41. PubMed PMID: 11037817.
Cao D, Begum S, Ali SZ, Westra WH. Expression of p16 in benign and malignant cystic squamous lesions of the neck. Hum Pathol. 2010 Apr;41(4):535-9. doi: 10.1016/j.humpath.2009.09.006. Epub 2009 Dec 11. PubMed PMID: 20004950.
Fakhry C, Lacchetti C, Rooper LM, Jordan RC, Rischin D, Sturgis EM, Bell D, Lingen MW, Harichand-Herdt S, Thibo J, Zevallos J, Perez-Ordonez B. Human Papillomavirus Testing in Head and Neck Carcinomas: ASCO Clinical Practice Guideline Endorsement of the College of American Pathologists Guideline. J Clin Oncol. 2018 Sep 6:JCO1800684. doi: 10.1200/JCO.18.00684. [Epub ahead of print] PubMed PMID: 30188786.
Presented by Dr. White and prepared by Austin McCuiston
40 year old male with a superficial inguinal mass.
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40 year old male with a superficial inguinal mass.
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Answer: D
Histology: Histologic sections show a round to ovoid, well circumscribed, variably cellular spindle cell lesion. The nuclei are ovoid to tapered, fairly uniform, and cytologically bland. The stroma in this case is dense and collagenous, and there are admixed lymphocytes, plasma cells, and mast cells. Numerous and prominent thick walled, small to medium caliber blood vessels are seen.
Discussion: Cellular angiofibromas are mesenchymal neoplasms which are typically superficial and arise in the vulvovaginal, perineal, and inguino-scrotal regions. Most lesions show the histologic features observed in this case, although some may show myxoid changes or adipocytic components.
Iwasa et al reported up to 60% of cellular angiofibromas are positive CD34, with a smaller percentage of tumors labeling positive for SMA and desmin. Positivity for ER and PR may be seen in cellular angiofibromas in both female and male patients, as demonstrated in this lesion from a male patient. S100 and cytokeratins are typically negative, and Demicco et al more recently demonstrated cellular angiofibromas are also negative for STAT6.
Cellular angiofibromas show overlapping morphologic features with spindle cell lipomas and mammary-type myofibroblastomas. More recently deletions on chromosome 13q14 have also been described in cellular angiofibromas with monoallelic deletions of RB1 detected by FISH, suggesting these entities are genetically similar.
References:
Iwasa Y, Fletcher CD. Cellular angiofibroma: clinicopathologic and immunohistochemical analysis of 51 cases. Am J Surg Pathol. 2004 Nov;28(11):1426-35. PubMed PMID: 15489646.
Demicco EG, Harms PW, Patel RM, et al. Extensive survey of STAT6 expression in a large series of mesenchymal tumors. Am J Clin Pathol. 2015;143(5):672-82.
Flucke U, van Krieken JH, Mentzel T. Cellular angiofibroma: analysis of 25 cases emphasizing its relationship to spindle cell lipoma and mammary-type myofibroblastoma. Mod Pathol. 2011 Jan;24(1):82-9. doi: 10.1038/modpathol.2010.170. Epub 2010 Sep 17. PubMed PMID: 20852591.
Incorrect
Answer: D
Histology: Histologic sections show a round to ovoid, well circumscribed, variably cellular spindle cell lesion. The nuclei are ovoid to tapered, fairly uniform, and cytologically bland. The stroma in this case is dense and collagenous, and there are admixed lymphocytes, plasma cells, and mast cells. Numerous and prominent thick walled, small to medium caliber blood vessels are seen.
Discussion: Cellular angiofibromas are mesenchymal neoplasms which are typically superficial and arise in the vulvovaginal, perineal, and inguino-scrotal regions. Most lesions show the histologic features observed in this case, although some may show myxoid changes or adipocytic components.
Iwasa et al reported up to 60% of cellular angiofibromas are positive CD34, with a smaller percentage of tumors labeling positive for SMA and desmin. Positivity for ER and PR may be seen in cellular angiofibromas in both female and male patients, as demonstrated in this lesion from a male patient. S100 and cytokeratins are typically negative, and Demicco et al more recently demonstrated cellular angiofibromas are also negative for STAT6.
Cellular angiofibromas show overlapping morphologic features with spindle cell lipomas and mammary-type myofibroblastomas. More recently deletions on chromosome 13q14 have also been described in cellular angiofibromas with monoallelic deletions of RB1 detected by FISH, suggesting these entities are genetically similar.
References:
Iwasa Y, Fletcher CD. Cellular angiofibroma: clinicopathologic and immunohistochemical analysis of 51 cases. Am J Surg Pathol. 2004 Nov;28(11):1426-35. PubMed PMID: 15489646.
Demicco EG, Harms PW, Patel RM, et al. Extensive survey of STAT6 expression in a large series of mesenchymal tumors. Am J Clin Pathol. 2015;143(5):672-82.
Flucke U, van Krieken JH, Mentzel T. Cellular angiofibroma: analysis of 25 cases emphasizing its relationship to spindle cell lipoma and mammary-type myofibroblastoma. Mod Pathol. 2011 Jan;24(1):82-9. doi: 10.1038/modpathol.2010.170. Epub 2010 Sep 17. PubMed PMID: 20852591.
Presented by Dr. White and prepared by Austin McCuiston.
60 year old female with an intracardiac tumor.
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60 year old female with an intracardiac tumor.
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Answer: D
Histology: Histologic sections showed a paucicellular polypoid lesion, with scattered stellate to small round cells embedded in a fibromyxoid stroma with associated fibrin. In focal areas, the lesional cells form small clusters, cords, and gland-like structures.
Discussion:
Cardiac myxomas are the most common primary cardiac tumor. However, cardiac myxomas only represent a small fraction of all cardiac tumors, which are most frequently metastases. Cardiac myxomas typically arise in the left atrium, and form peduculated or polypoid lobulated masses. Histologically, they have abundant fibromyxoid stroma, although areas of hemorrhage or cystic degeneration may be seen. The neoplastic cells are stellate to round and may form small clusters or glandular structures. These cells label positively for vascular immunostains (CD31 and CD34) and calretinin. Additionally, the glandular cells may label for cytokeratins.
Cardiac myxomas are most frequently sporadic. However, they may be seen in the setting of Carney’s complex, an autosomal dominant multiple neoplasia syndrome conferring an increased risk for cardiac and extra-cardiac myxomas, psammomatous melanotic schwannomas, multiple lentigines, epthelioid blue nevi, large cell calcifying sertoli cell tumor (LCCST), and pituitary adenomas. It is also associated with endocrine over reactivity such as primary pigmented nodular adrenocortical disease (PPNAD). Mutations in protein kinase A regulatory subunit 1 alpha (PRKAR1A) have been identified in both sporadic and familial cases of Carney complex.
Although benign, cardiac myxomas may behave in a clinically aggressive or “malignant” fashion where fragments of the tumor may detach and embolize to the brain and/or other sites, or the tumor mass itself may cause cardiac outflow obstruction.
Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac tumours: diagnosis and management. Lancet Oncol. 2005 Apr;6(4):219-28. Review. PubMed PMID: 15811617.
Incorrect
Answer: D
Histology: Histologic sections showed a paucicellular polypoid lesion, with scattered stellate to small round cells embedded in a fibromyxoid stroma with associated fibrin. In focal areas, the lesional cells form small clusters, cords, and gland-like structures.
Discussion:
Cardiac myxomas are the most common primary cardiac tumor. However, cardiac myxomas only represent a small fraction of all cardiac tumors, which are most frequently metastases. Cardiac myxomas typically arise in the left atrium, and form peduculated or polypoid lobulated masses. Histologically, they have abundant fibromyxoid stroma, although areas of hemorrhage or cystic degeneration may be seen. The neoplastic cells are stellate to round and may form small clusters or glandular structures. These cells label positively for vascular immunostains (CD31 and CD34) and calretinin. Additionally, the glandular cells may label for cytokeratins.
Cardiac myxomas are most frequently sporadic. However, they may be seen in the setting of Carney’s complex, an autosomal dominant multiple neoplasia syndrome conferring an increased risk for cardiac and extra-cardiac myxomas, psammomatous melanotic schwannomas, multiple lentigines, epthelioid blue nevi, large cell calcifying sertoli cell tumor (LCCST), and pituitary adenomas. It is also associated with endocrine over reactivity such as primary pigmented nodular adrenocortical disease (PPNAD). Mutations in protein kinase A regulatory subunit 1 alpha (PRKAR1A) have been identified in both sporadic and familial cases of Carney complex.
Although benign, cardiac myxomas may behave in a clinically aggressive or “malignant” fashion where fragments of the tumor may detach and embolize to the brain and/or other sites, or the tumor mass itself may cause cardiac outflow obstruction.
Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac tumours: diagnosis and management. Lancet Oncol. 2005 Apr;6(4):219-28. Review. PubMed PMID: 15811617.
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