Presented by Dr. Lisa Rooper and prepared by Dr. Tatianna Larman.
A 70 year old woman with a nasal obstruction.
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A 70 year old female with a nasal obstruction. Choose the correct diagnosis.
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Answer: Sinonasal papilloma, oncocytic (cylindrical cell) type
Histology: The tumor demonstrates both inverted and exophytic growth. It consists of a proliferation of cuboidal to columnar oncocytic cells with abundant granular cytoplasm and round nuclei with variably prominent nucleoli. The cells are arranged in multiple layers with prominent intraepithelial mucous cysts and microabscesses. These findings are consistent with a sinonasal papilloma, oncocytic (cylindrical cell) type.
Discussion: Sinonasal papillomas, previously known as schneiderian papillomas, can be divided into three subtypes: inverted, exophytic, and oncocytic. Of these, the oncocytic subtype is the least common. Despite both inverted and exophytic growth, the prominent oncocytic cytology sets oncocytic papillomas apart from inverted and exophytic papillomas. Like inverted papillomas, oncocytic papillomas most commonly arise on the lateral nasal wall and are associated with an approximately 5% chance of malignant transformation. Although the role of HPV remains controversial in sinonasal papillomas in general, the oncocytic subtype is not thought to be associated with either low-risk or high-risk HPV. Recently, activating KRAS mutations have been reported in a large proportion of these tumors.
Incorrect
Answer: Sinonasal papilloma, oncocytic (cylindrical cell) type
Histology: The tumor demonstrates both inverted and exophytic growth. It consists of a proliferation of cuboidal to columnar oncocytic cells with abundant granular cytoplasm and round nuclei with variably prominent nucleoli. The cells are arranged in multiple layers with prominent intraepithelial mucous cysts and microabscesses. These findings are consistent with a sinonasal papilloma, oncocytic (cylindrical cell) type.
Discussion: Sinonasal papillomas, previously known as schneiderian papillomas, can be divided into three subtypes: inverted, exophytic, and oncocytic. Of these, the oncocytic subtype is the least common. Despite both inverted and exophytic growth, the prominent oncocytic cytology sets oncocytic papillomas apart from inverted and exophytic papillomas. Like inverted papillomas, oncocytic papillomas most commonly arise on the lateral nasal wall and are associated with an approximately 5% chance of malignant transformation. Although the role of HPV remains controversial in sinonasal papillomas in general, the oncocytic subtype is not thought to be associated with either low-risk or high-risk HPV. Recently, activating KRAS mutations have been reported in a large proportion of these tumors.
Presented by Dr. Lisa Rooper and prepared by Dr. Tatianna Larman.
A 30 year old woman with a thyroid nodule.
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A 30 year old woman with a thyroid nodule. Choose the correct diagnosis.
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Answer: Papillary carcinoma, oncocytic variant
Histology: The tumor consists of a proliferation of eosinophilic cells with abundant granular cytoplasm and papillary architecture. Although many of the tumor cells demonstrate coarse chromatin with central nucleoli, consistent with an oncocytic neoplasm, they also show nuclear features suggestive of papillary carcinoma, including marked membrane irregularity, prominent nuclear grooves, and patchy chromatin clearing. Taken in sum, these findings are consistent with a diagnosis of oncocytic variant of papillary carcinoma.
Discussion: Oncocytic variant of papillary thyroid carcinoma makes up less than 10% of all papillary carcinomas. They can demonstrate either papillary or follicular architecture. The papillary nuclear features can be relatively subtle in these tumors compared to other subtypes of papillary carcinoma, with only focal nuclear clearing, nuclear grooves, and nuclear pseudoinclusions. However, this atypia should be more marked than what is acceptable for true Hurthle cell neoplasms. The main differential diagnosis for oncocytic variant of papillary carcinoma is the tall-cell variant of papillary carcinoma, which should have more prominent papillary nuclear features and cells that are 3 times as tall as they are wide. It is essential to make this distinction because the oncocytic variant lacks the aggressive behavior of tall cell variant and instead has a risk profile similar to conventional papillary carcinoma.
Incorrect
Answer: Papillary carcinoma, oncocytic variant
Histology: The tumor consists of a proliferation of eosinophilic cells with abundant granular cytoplasm and papillary architecture. Although many of the tumor cells demonstrate coarse chromatin with central nucleoli, consistent with an oncocytic neoplasm, they also show nuclear features suggestive of papillary carcinoma, including marked membrane irregularity, prominent nuclear grooves, and patchy chromatin clearing. Taken in sum, these findings are consistent with a diagnosis of oncocytic variant of papillary carcinoma.
Discussion: Oncocytic variant of papillary thyroid carcinoma makes up less than 10% of all papillary carcinomas. They can demonstrate either papillary or follicular architecture. The papillary nuclear features can be relatively subtle in these tumors compared to other subtypes of papillary carcinoma, with only focal nuclear clearing, nuclear grooves, and nuclear pseudoinclusions. However, this atypia should be more marked than what is acceptable for true Hurthle cell neoplasms. The main differential diagnosis for oncocytic variant of papillary carcinoma is the tall-cell variant of papillary carcinoma, which should have more prominent papillary nuclear features and cells that are 3 times as tall as they are wide. It is essential to make this distinction because the oncocytic variant lacks the aggressive behavior of tall cell variant and instead has a risk profile similar to conventional papillary carcinoma.
Presented by Dr. Lisa Rooper and prepared by Dr. Tatianna Larman.
An 80 year old female with a neck mass.
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An 80 year old woman with a neck mass. Choose the correct diagnosis.
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Answer: Secretory carcinoma with tumor-associated lymphoid proliferation
Histology: The parotid tumor consists of a cystic proliferation of oncocytic cells with prominent lymphoid stroma. Although these features are suggestive of a Warthin tumor, the tumor lacks the classic bilayered cell population and round nuclei with prominent nucleoli diagnostic of Warthin tumor. Instead, the cysts are lined by a monolayer of oncocytic cells with abundant vacuolated cytoplasm and vesicular nuclei. In a few foci, these cell coalesce into a hypercellular proliferation with prominent microcystic architecture and intraluminal secretions. These findings are characteristic of secretory carcinoma, which in this case has a prominent tumor-associated lymphoid proliferation.
Discussion: Secretory carcinoma, also known as mammary analogue secretory carcinoma, was described as a unique salivary gland malignancy in 2010. Most of these tumors were previously categorized as papillary cystic variant of acinic cell carcinoma, but were recognized as an independent entity based on their similarity to secretory carcinoma of the breast and recurrent ETV6-NTRK3 gene rearrangements. Secretory carcinomas demonstrate cystic, microcystic, and papillary architecture and immunohistochemical positivity for S100 and mammoglobin. They lack the true zymogen granule formation seen in acinic cell carcinomas. Although they may demonstrate luminal mucin, they also lack the true goblet cells of mucoepidermoid carcinoma. As with many salivary gland tumors, secretory carcinoma can show prominent associated lymphoid response, known as tumor-associated lymphoid proliferation. This finding has minimal prognostic significance but should not be misinterpreted as a lymph node metastasis or part of a Warthin tumor.
Incorrect
Answer: Secretory carcinoma with tumor-associated lymphoid proliferation
Histology: The parotid tumor consists of a cystic proliferation of oncocytic cells with prominent lymphoid stroma. Although these features are suggestive of a Warthin tumor, the tumor lacks the classic bilayered cell population and round nuclei with prominent nucleoli diagnostic of Warthin tumor. Instead, the cysts are lined by a monolayer of oncocytic cells with abundant vacuolated cytoplasm and vesicular nuclei. In a few foci, these cell coalesce into a hypercellular proliferation with prominent microcystic architecture and intraluminal secretions. These findings are characteristic of secretory carcinoma, which in this case has a prominent tumor-associated lymphoid proliferation.
Discussion: Secretory carcinoma, also known as mammary analogue secretory carcinoma, was described as a unique salivary gland malignancy in 2010. Most of these tumors were previously categorized as papillary cystic variant of acinic cell carcinoma, but were recognized as an independent entity based on their similarity to secretory carcinoma of the breast and recurrent ETV6-NTRK3 gene rearrangements. Secretory carcinomas demonstrate cystic, microcystic, and papillary architecture and immunohistochemical positivity for S100 and mammoglobin. They lack the true zymogen granule formation seen in acinic cell carcinomas. Although they may demonstrate luminal mucin, they also lack the true goblet cells of mucoepidermoid carcinoma. As with many salivary gland tumors, secretory carcinoma can show prominent associated lymphoid response, known as tumor-associated lymphoid proliferation. This finding has minimal prognostic significance but should not be misinterpreted as a lymph node metastasis or part of a Warthin tumor.
Presented by Dr. Jonathan Epstein and prepared by Dr. Tatianna Larman.
A 67 year old female presented with hematuria and a bladder biopsy was performed. A Congo red stain was negative on several attempts.
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A 67 year old female presented with hematuria and a bladder biopsy was performed. A Congo red stain was negative on several attempts. Choose the correct diagnosis.
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Answer: Light chain deposition disease and plasma cell dyscrasia
Histology: Within the lamina propria are large deposits of amorphous pink material, some around blood vessels and others in the stroma. Focally, there is a giant cell reaction to the material. Surrounding the deposits is a brisk plasma cell infiltrate, which was shown immunohistochemically to be kappa light chain restricted.
Discussion: : In 99.99% of cases with this morphology, the diagnosis would be amyloidosis of the bladder with a high likelihood of associated plasma cell dyscrasia, since the usual case of amyloidosis in the bladder does not have many plasma cells. Amyloid should stain orange with Congo red and also should appear green under polarized light. In this case despite repeated staining, the amorphous deposits failed to stain for Congo red. The plasma cells were almost all kappa light chain restricted consistent with a plasma cell dyscrasia. These findings are classic for light chain deposition disease. Light chain deposition disease is a rare systemic disorder with deposition of mostly monoclonal amorphous nonamyloid light chains in multiple organs. In 80% of cases, kappa light chain is involved. Renal involvement with rapidly progressing renal failure is the dominant manifestation of the disease with liver and heart involvement the next most common sites. Approximately 50% of cases are associated either concurrently or subsequently with plasma cell dyscrasia, so that patients have to be worked up for multiple myeloma. In an additional 30%-40% of cases, a plasma cell dyscrasia/monoclonal gammopathy of uncertain significance is diagnosed. In up to 10% of patients, no monoclonal proteins are detectable in serum or urine. Bladder involvement with this disease has not to my knowledge been reported.
Incorrect
Answer: Light chain deposition disease and plasma cell dyscrasia
Histology: Within the lamina propria are large deposits of amorphous pink material, some around blood vessels and others in the stroma. Focally, there is a giant cell reaction to the material. Surrounding the deposits is a brisk plasma cell infiltrate, which was shown immunohistochemically to be kappa light chain restricted.
Discussion: : In 99.99% of cases with this morphology, the diagnosis would be amyloidosis of the bladder with a high likelihood of associated plasma cell dyscrasia, since the usual case of amyloidosis in the bladder does not have many plasma cells. Amyloid should stain orange with Congo red and also should appear green under polarized light. In this case despite repeated staining, the amorphous deposits failed to stain for Congo red. The plasma cells were almost all kappa light chain restricted consistent with a plasma cell dyscrasia. These findings are classic for light chain deposition disease. Light chain deposition disease is a rare systemic disorder with deposition of mostly monoclonal amorphous nonamyloid light chains in multiple organs. In 80% of cases, kappa light chain is involved. Renal involvement with rapidly progressing renal failure is the dominant manifestation of the disease with liver and heart involvement the next most common sites. Approximately 50% of cases are associated either concurrently or subsequently with plasma cell dyscrasia, so that patients have to be worked up for multiple myeloma. In an additional 30%-40% of cases, a plasma cell dyscrasia/monoclonal gammopathy of uncertain significance is diagnosed. In up to 10% of patients, no monoclonal proteins are detectable in serum or urine. Bladder involvement with this disease has not to my knowledge been reported.
Presented by Dr. Jonathan Epstein and prepared by Dr. Tatianna Larman.
A cystectomy specimen in a 55 year old man.
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A cystectomy specimen in a 55 year old man. Choose the correct diagnosis.
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Answer: Nested variant of urothelial carcinoma
Histology: Large irregular nests of urothelium extend deep into the muscularis propria and focally into the perivesicular soft tissue. Cytologically, the urothelial cells are extremely bland, some with nuclear grooves as seen in normal urothelium, and virtually no mitotic activity. In general, there is little to no stromal reaction around the tumor nests. On the surface there is a focal exophytic papillary component.
Discussion: There is a general rule in bladder pathology that if you see nests of urothelium in the muscularis propria then it has to be invasive urothelial carcinoma. Mimickers of invasive urothelial carcinoma, such a florid proliferation of von Brun nests or inverted papilloma can be large and fill the lamina propria but not extend into the muscularis propria. The only exceptions are non-tumoral, such as in a diverticulum, urachal remnant, and the intramural portion of the ureters. Similarly, inverted growth patterns of PUNLMP or non-invasive low grade papillary urothelial carcinoma do not invade the muscularis propria. The findings in this case are typical of nested urothelial carcinoma, and in particular the large nested variant. The hallmark of nested carcinoma is the bland cytology indistinguishable in most areas of the tumor from normal urothelium. Architecturally, typical nested carcinoma consists of small nests that are too crowded or infiltrate irregularly in the lamina propria to be von Brun nests. When nested carcinoma is seen in the muscularis propria, the diagnosis is more straightforward since as noted above von Brun nests do not extend into the muscularis propria. The large nested variant consists of much larger nests that mimic the inverted growth pattern on non-invasive PUNLMP or low grade papillary urothelial carcinoma. However, if nests of urothelium are seen infiltrating between bundles of the muscularis propria no matter how cytologically bland, then the diagnosis is nested carcinoma. It is very difficult to diagnose large nested carcinoma on a biopsy where muscularis propria invasion is not seen. In these cases the irregular and haphazard arrangement of the nests provide the clues to its malignancy. In most cases of nested carcinoma there is no surface precursor component which adds to the difficulty in establishing a diagnosis. Nested carcinoma, stage for stage, behaves the same as infiltrating high grade urothelial carcinoma.
References:
Large nested variant of urothelial carcinoma: 23 cases mimicking von Brunn nests and inverted growth pattern of noninvasive papillary urothelial carcinoma. Cox R, Epstein JI. Am J Surg Pathol. 2011 Sep;35(9):1337-42.
Florid von Brunn nests mimicking urothelial carcinoma: a morphologic and immunohistochemical comparison to the nested variant of urothelial carcinoma. Volmar KE, Chan TY, De Marzo AM, Epstein JI. Am J Surg Pathol. 2003 Sep;27(9):1243-52.
Incorrect
Answer: Nested variant of urothelial carcinoma
Histology: Large irregular nests of urothelium extend deep into the muscularis propria and focally into the perivesicular soft tissue. Cytologically, the urothelial cells are extremely bland, some with nuclear grooves as seen in normal urothelium, and virtually no mitotic activity. In general, there is little to no stromal reaction around the tumor nests. On the surface there is a focal exophytic papillary component.
Discussion: There is a general rule in bladder pathology that if you see nests of urothelium in the muscularis propria then it has to be invasive urothelial carcinoma. Mimickers of invasive urothelial carcinoma, such a florid proliferation of von Brun nests or inverted papilloma can be large and fill the lamina propria but not extend into the muscularis propria. The only exceptions are non-tumoral, such as in a diverticulum, urachal remnant, and the intramural portion of the ureters. Similarly, inverted growth patterns of PUNLMP or non-invasive low grade papillary urothelial carcinoma do not invade the muscularis propria. The findings in this case are typical of nested urothelial carcinoma, and in particular the large nested variant. The hallmark of nested carcinoma is the bland cytology indistinguishable in most areas of the tumor from normal urothelium. Architecturally, typical nested carcinoma consists of small nests that are too crowded or infiltrate irregularly in the lamina propria to be von Brun nests. When nested carcinoma is seen in the muscularis propria, the diagnosis is more straightforward since as noted above von Brun nests do not extend into the muscularis propria. The large nested variant consists of much larger nests that mimic the inverted growth pattern on non-invasive PUNLMP or low grade papillary urothelial carcinoma. However, if nests of urothelium are seen infiltrating between bundles of the muscularis propria no matter how cytologically bland, then the diagnosis is nested carcinoma. It is very difficult to diagnose large nested carcinoma on a biopsy where muscularis propria invasion is not seen. In these cases the irregular and haphazard arrangement of the nests provide the clues to its malignancy. In most cases of nested carcinoma there is no surface precursor component which adds to the difficulty in establishing a diagnosis. Nested carcinoma, stage for stage, behaves the same as infiltrating high grade urothelial carcinoma.
References:
Large nested variant of urothelial carcinoma: 23 cases mimicking von Brunn nests and inverted growth pattern of noninvasive papillary urothelial carcinoma. Cox R, Epstein JI. Am J Surg Pathol. 2011 Sep;35(9):1337-42.
Florid von Brunn nests mimicking urothelial carcinoma: a morphologic and immunohistochemical comparison to the nested variant of urothelial carcinoma. Volmar KE, Chan TY, De Marzo AM, Epstein JI. Am J Surg Pathol. 2003 Sep;27(9):1243-52.
Presented by Dr. Jonathan Epstein and prepared by Dr. Tatianna Larman.
A 65 year old man presents with a testicular mass.
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A 65 year old man presents with a testicular mass. Choose the correct diagnosis.
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Answer: Metastatic prostate adenocarcinoma
Histology: Within a testis that has a small rim of atrophic seminiferous tubules is a large mass composed of sheets of cells. In areas there is a papillary appearance to the tumor with necrosis between the fronds and necrotic cells lining the fronds. In areas of the tumor the cytology is relatively uniform consisting of large cells with abundant cytoplasm and round to oval nuclei with central prominent nucleoli. A minor component of the tumor consists of much more pleomorphic cells with marked variation in nuclear size and shape.
Discussion: The papillary structures seen in this case are artifactual. In between the “papillary fronds” is necrosis and the cells lining the “papillary” structures have necrotic cells and apoptosis on the surface. With necrosis away further from the blood supply it leaves residual viable cancer around the capillaries mimicking a papillary structure. True papillary fronds would not have intervening necrosis between fronds and would not have necrotic cells on the outer surface of the fronds. Embryonal carcinoma cells are much more pleomorphic with multiple irregular clumps of chromatin compared to the uniform population of round to oval nuclei with a single central nucleolus seen in this case. Embryonal carcinoma would also in areas show primitive luminal formation. Seminoma consists of loosely cohesive cells as opposed to the compact sheets of tumor in this case. Yolk sac tumors can on occasion show diffuse solid growth, which typically mimic seminoma. On one hand, yolk sac tumor cytology is more pleomorphic than the areas of the current case with uniform cytology, and on the other hand would not show the pleomorphism focally present in the current case. The uniform less cytologically atypical component of this tumor is typical of what one could see in metastatic prostate adenocarcinoma. What is unusual in this case is the more pleomorphic component that one typically does not associate with prostate cancer. However, there is the pleomorphic giant cell variant of prostate adenocarcinoma which is composed of Gleason score 9-10 usual acinar carcinoma with a minor component of bizarre pleomorphic tumor cells not usually seen in prostate cancer. Initially reported on only 6 cases, we are in the process of analyzing a series of 22 cases with a broader panel of immunohistochemistry. In order to prove the prostatic origin of the tumor, immunohistochemistry for NKX3.1 is much more sensitive than PSA or P501s (Prostein), where the latter two can give rise to false negative staining. Prostate cancer is the most common cancer to metastasize to the testis, typically in the setting of widespread metastatic disease.
References:
Pseudopapillary features in prostatic adenocarcinoma mimicking urothelial carcinoma: a diagnostic pitfall. Gordetsky J, Epstein JI. Am J Surg Pathol. 2014 Jul;38(7):941-5.
Pleomorphic giant cell adenocarcinoma of the prostate: report of 6 cases. Parwani AV, Herawi M, Epstein JI. Am J Surg Pathol. 2006 Oct;30(10):1254-9
Incorrect
Answer: Metastatic prostate adenocarcinoma
Histology: Within a testis that has a small rim of atrophic seminiferous tubules is a large mass composed of sheets of cells. In areas there is a papillary appearance to the tumor with necrosis between the fronds and necrotic cells lining the fronds. In areas of the tumor the cytology is relatively uniform consisting of large cells with abundant cytoplasm and round to oval nuclei with central prominent nucleoli. A minor component of the tumor consists of much more pleomorphic cells with marked variation in nuclear size and shape.
Discussion: The papillary structures seen in this case are artifactual. In between the “papillary fronds” is necrosis and the cells lining the “papillary” structures have necrotic cells and apoptosis on the surface. With necrosis away further from the blood supply it leaves residual viable cancer around the capillaries mimicking a papillary structure. True papillary fronds would not have intervening necrosis between fronds and would not have necrotic cells on the outer surface of the fronds. Embryonal carcinoma cells are much more pleomorphic with multiple irregular clumps of chromatin compared to the uniform population of round to oval nuclei with a single central nucleolus seen in this case. Embryonal carcinoma would also in areas show primitive luminal formation. Seminoma consists of loosely cohesive cells as opposed to the compact sheets of tumor in this case. Yolk sac tumors can on occasion show diffuse solid growth, which typically mimic seminoma. On one hand, yolk sac tumor cytology is more pleomorphic than the areas of the current case with uniform cytology, and on the other hand would not show the pleomorphism focally present in the current case. The uniform less cytologically atypical component of this tumor is typical of what one could see in metastatic prostate adenocarcinoma. What is unusual in this case is the more pleomorphic component that one typically does not associate with prostate cancer. However, there is the pleomorphic giant cell variant of prostate adenocarcinoma which is composed of Gleason score 9-10 usual acinar carcinoma with a minor component of bizarre pleomorphic tumor cells not usually seen in prostate cancer. Initially reported on only 6 cases, we are in the process of analyzing a series of 22 cases with a broader panel of immunohistochemistry. In order to prove the prostatic origin of the tumor, immunohistochemistry for NKX3.1 is much more sensitive than PSA or P501s (Prostein), where the latter two can give rise to false negative staining. Prostate cancer is the most common cancer to metastasize to the testis, typically in the setting of widespread metastatic disease.
References:
Pseudopapillary features in prostatic adenocarcinoma mimicking urothelial carcinoma: a diagnostic pitfall. Gordetsky J, Epstein JI. Am J Surg Pathol. 2014 Jul;38(7):941-5.
Pleomorphic giant cell adenocarcinoma of the prostate: report of 6 cases. Parwani AV, Herawi M, Epstein JI. Am J Surg Pathol. 2006 Oct;30(10):1254-9
Presented by Dr. Andres Matoso and prepared by Dr. Tatianna Larman.
An 84 year old woman with a urethral lesion.
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An 84 year old woman with a urethral lesion. Choose the correct diagnosis.
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Answer: Urethral melanoma
Histology: Ulcerated polypoid lesion with highly atypical cells with marked nuclear pleomorphism, prominent nucleoli and brisk mitotic activity. There are areas of spindle morphology mimicking a high-grade sarcoma or sarcomatoid carcinoma. There are areas of focal pigment that is difficult to differentiate between melanin and hemosiderin.
Discussion: Primary urethral melanoma is rare and comprises 0.2% of all melanomas and 4% of all urethral primary malignancies. Older women are most frequently affected and the distal urethra is involved in the majority of cases. Primary malignant melanomas of the urethra, one fifth of which are amelanotic, must be included in the differential diagnosis of a number of primary neoplasms that involve the urethra, including urothelial carcinoma, sarcomatoid carcinoma, and sarcomas. The high-grade features and, in some cases, the presence of pigment should suggest the diagnosis. Immunohistochemistry for melanocytic markers are usually needed for a definitive diagnosis.
References:
1. Int Urogynecol J (2015) 26:149–150
2. Kim CJ, Pak K, Hamaguchi A, Ishida A, Arai Y, Konishi T, et al. Primary malignant melanoma of the female urethra. Cancer 1993;71:448-51.
3. Oliva E, Quinn TR, Amin MB, Eble JN, Epstein JI, Srigley JR, et al. Primary malignant melanoma of the urethra: A clinicopathologic analysis of 15 cases. Am J Surg Pathol 2000;24:785-96.
Incorrect
Answer: Urethral melanoma
Histology: Ulcerated polypoid lesion with highly atypical cells with marked nuclear pleomorphism, prominent nucleoli and brisk mitotic activity. There are areas of spindle morphology mimicking a high-grade sarcoma or sarcomatoid carcinoma. There are areas of focal pigment that is difficult to differentiate between melanin and hemosiderin.
Discussion: Primary urethral melanoma is rare and comprises 0.2% of all melanomas and 4% of all urethral primary malignancies. Older women are most frequently affected and the distal urethra is involved in the majority of cases. Primary malignant melanomas of the urethra, one fifth of which are amelanotic, must be included in the differential diagnosis of a number of primary neoplasms that involve the urethra, including urothelial carcinoma, sarcomatoid carcinoma, and sarcomas. The high-grade features and, in some cases, the presence of pigment should suggest the diagnosis. Immunohistochemistry for melanocytic markers are usually needed for a definitive diagnosis.
References:
1. Int Urogynecol J (2015) 26:149–150
2. Kim CJ, Pak K, Hamaguchi A, Ishida A, Arai Y, Konishi T, et al. Primary malignant melanoma of the female urethra. Cancer 1993;71:448-51.
3. Oliva E, Quinn TR, Amin MB, Eble JN, Epstein JI, Srigley JR, et al. Primary malignant melanoma of the urethra: A clinicopathologic analysis of 15 cases. Am J Surg Pathol 2000;24:785-96.
Histology: There is a cellular proliferation composed of elongated and plump cells infiltrating the bladder wall forming intersecting fascicles. There is an edematous or myxoid background with acute and chronic inflammation, extravasated red blood cells and a network of small blood vessels. Mitoses are seen but are not frequent and there are no atypical mitoses. The overlying urothelium is benign and there is submucosal edema and inflammation.
Discussion: Inflammatroy myofibroblastic tumor of the bladder can arise in different clinical scenarios including post-operative nodules or spontaneously de-novo. The main differential diagnoses include leiomyosarcoma, and sarcomatoid carcinoma. Sarcomatoid carcinoma usually has more significant atypia and an epithelial component is usually present. Immunohistochemistry should be interpreted in the overall context of the lesion as there is significant overlap in the results. While diffuse staining for cytokeratins including CK5/6 and high molecular weight cytokeratins would favor sarcomatoid carcinoma, myofibroblast can often also display keratin staining. Diffuse staining for desmin and SMA in the absence of other markers would favor leiomyosarcoma. Approximately 60% of these tumors may show overexpression of ALK by immunohistochemistry, which can be confirmed by FISH. In small biopsies, caution should be exercised when diagnosing an IMT because similar stromal reaction can be seen adjacent to another tumor.
Histology: There is a cellular proliferation composed of elongated and plump cells infiltrating the bladder wall forming intersecting fascicles. There is an edematous or myxoid background with acute and chronic inflammation, extravasated red blood cells and a network of small blood vessels. Mitoses are seen but are not frequent and there are no atypical mitoses. The overlying urothelium is benign and there is submucosal edema and inflammation.
Discussion: Inflammatroy myofibroblastic tumor of the bladder can arise in different clinical scenarios including post-operative nodules or spontaneously de-novo. The main differential diagnoses include leiomyosarcoma, and sarcomatoid carcinoma. Sarcomatoid carcinoma usually has more significant atypia and an epithelial component is usually present. Immunohistochemistry should be interpreted in the overall context of the lesion as there is significant overlap in the results. While diffuse staining for cytokeratins including CK5/6 and high molecular weight cytokeratins would favor sarcomatoid carcinoma, myofibroblast can often also display keratin staining. Diffuse staining for desmin and SMA in the absence of other markers would favor leiomyosarcoma. Approximately 60% of these tumors may show overexpression of ALK by immunohistochemistry, which can be confirmed by FISH. In small biopsies, caution should be exercised when diagnosing an IMT because similar stromal reaction can be seen adjacent to another tumor.
Presented by Dr. Andrew Matoso and prepared by Dr. Tatianna Larman.
A 37 year old man with a bladder tumor.
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A 37 year old man with a bladder tumor. Choose the correct diagnosis.
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Answer: BLADDER PARAGANGLIOMA
Histology: Nests of tumor cells separated by thin delicate plexiform vascular network and fibrous septa. Cells display an amphophilic cytoplasm with visible nucleoli. Nuclei can display large bizarre atypia, degenerative type. The tumor involves the muscularis propria.
Discussion: Paragangliomas of the urinary bladder account for less than 0.5% of bladder tumors. The majority of the patients experience micturition attacks. Filling of the bladder or urination can trigger headaches, blurred vision, palpitations and syncope. Elevated serum catecholamines can help in the diagnosis. By immunohistochemistry, tumor cells are positive for GATA3 which could contribute to a not infrequent misdiagnosis as urothelial carcinoma. Keratin stains should be negative and neuroendocrine markers are positive. Another frequent misdiagnosis is carcinoid tumor, however, carcinoid tumors in the bladder have a predominant tubular architecture which is not seen in paragangliomas. SDHB immunohistochemistry should be performed to diagnose SDH-deficient paragangliomas which are usually associated with a hereditary mutation in any of the subunits of SDH and often carry a malignant behavior.
Reference(s):
1. Clin Genitourin Cancer. 2017 Mar 28. pii: S1558-7673(17)30072-1.
2. Am J Surg Pathol. 2004 Jan;28(1):94-100.
3. Mod Pathol. 2013 Oct;26(10):1365-70. doi: 10.1038/modpathol.2013.76.
Incorrect
Answer: BLADDER PARAGANGLIOMA
Histology: Nests of tumor cells separated by thin delicate plexiform vascular network and fibrous septa. Cells display an amphophilic cytoplasm with visible nucleoli. Nuclei can display large bizarre atypia, degenerative type. The tumor involves the muscularis propria.
Discussion: Paragangliomas of the urinary bladder account for less than 0.5% of bladder tumors. The majority of the patients experience micturition attacks. Filling of the bladder or urination can trigger headaches, blurred vision, palpitations and syncope. Elevated serum catecholamines can help in the diagnosis. By immunohistochemistry, tumor cells are positive for GATA3 which could contribute to a not infrequent misdiagnosis as urothelial carcinoma. Keratin stains should be negative and neuroendocrine markers are positive. Another frequent misdiagnosis is carcinoid tumor, however, carcinoid tumors in the bladder have a predominant tubular architecture which is not seen in paragangliomas. SDHB immunohistochemistry should be performed to diagnose SDH-deficient paragangliomas which are usually associated with a hereditary mutation in any of the subunits of SDH and often carry a malignant behavior.
Reference(s):
1. Clin Genitourin Cancer. 2017 Mar 28. pii: S1558-7673(17)30072-1.
2. Am J Surg Pathol. 2004 Jan;28(1):94-100.
3. Mod Pathol. 2013 Oct;26(10):1365-70. doi: 10.1038/modpathol.2013.76.
Presented by Dr. Lisa Rooper and prepared by Dr. Tatianna Larman.
A 45 year old man underwent partial mandibulectomy for a jaw mass.
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A 45 year old man underwent partial mandibulectomy for a jaw mass. Choose the correct diagnosis.
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Answer: PERIPHERAL MUCOEPIDERMOID CARCINOMA WITH BONE INVASION
Histology: The bony cortex is replaced by an expansile cystic mass lined by three intermixed cell types: mucinous cells with intracytoplasmic mucin vacuoles, epidermoid cells with abundant cytoplasm and focal keratinization, and intermediate cells with minimal cytoplasm. In areas, these cells demonstrate complex architecture with invasion into the surrounding stroma.
Discussion: Mucoepidermoid carcinoma is the most common malignant salivary gland tumor. The majority of these tumors harbor a t(11;9) CRTC1-MAML2 fusion, which has been associated with a better prognosis. However, the main prognostic determinant is grade, which is assessed by the extent of a cystic component, degree of invasive growth, cytologic atypia, and necrosis. Mucoepidermoid carcinoma can involve the jaw bones by two routes: invasion from a peripheral tumor based in the gingiva or growth as a central lesion originating within the bone itself. In central lesions, the main differential diagnosis includes a glandular odontogenic cyst, a simple cyst with duct-like spaces, variable amounts of mucous cells, and lack of architectural complexity or invasive growth. Although glandular odontogenic cysts were once thought to be the precursors to central mucoepidermoid carcinomas, they have recently been shown to lack the CRTC1-MAML2 fusion. Peripheral tumors must be distinguished from adenosquamous carcinomas, which generally show separate superficial squamous components and deep glandular components rather than the intermixed cell types seen in mucoepidermoid carcinomas.
Incorrect
Answer: PERIPHERAL MUCOEPIDERMOID CARCINOMA WITH BONE INVASION
Histology: The bony cortex is replaced by an expansile cystic mass lined by three intermixed cell types: mucinous cells with intracytoplasmic mucin vacuoles, epidermoid cells with abundant cytoplasm and focal keratinization, and intermediate cells with minimal cytoplasm. In areas, these cells demonstrate complex architecture with invasion into the surrounding stroma.
Discussion: Mucoepidermoid carcinoma is the most common malignant salivary gland tumor. The majority of these tumors harbor a t(11;9) CRTC1-MAML2 fusion, which has been associated with a better prognosis. However, the main prognostic determinant is grade, which is assessed by the extent of a cystic component, degree of invasive growth, cytologic atypia, and necrosis. Mucoepidermoid carcinoma can involve the jaw bones by two routes: invasion from a peripheral tumor based in the gingiva or growth as a central lesion originating within the bone itself. In central lesions, the main differential diagnosis includes a glandular odontogenic cyst, a simple cyst with duct-like spaces, variable amounts of mucous cells, and lack of architectural complexity or invasive growth. Although glandular odontogenic cysts were once thought to be the precursors to central mucoepidermoid carcinomas, they have recently been shown to lack the CRTC1-MAML2 fusion. Peripheral tumors must be distinguished from adenosquamous carcinomas, which generally show separate superficial squamous components and deep glandular components rather than the intermixed cell types seen in mucoepidermoid carcinomas.
Presented by Dr. Lisa Rooper and prepared by Dr. Tatianna Larman.
A 40 year old woman presents for thyroid lobectomy after fine needle aspiration showed atypia of unknown significance.
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A 40 year old woman presents for thyroid lobectomy after fine needle aspiration showed atypia of unknown significance. Choose the correct diagnosis.
Correct
Answer: PARAGANGLIOMA
Histology: The tumor is actually centered outside the thyroid gland. It consists of a nested proliferation of cells with abundant amphophilic cytoplasm embedded in a variably vascular and fibrotic stroma. Nuclei show moderate variation in size with speckled chromatin and occasional prominent nucleoli. Tumor cells are positive for synaptophysin, INSM1, and GATA3, and negative for AE1/AE3 with retained SDHB. Sustentacular cells are positive for S100.
Discussion: In the head and neck, paragangliomas most commonly arise in the carotid body but can affect a wide range of sites, including within or adjacent to the thyroid gland. Because these tumors frequently arise from parasympathetic paraganglia, they are most often non-functional and generally present with mass effect. Historically, tumor behavior has been regarded as unpredictable, without good correlation to histologic features. However, most malignant paragangliomas have recently been associated with SDH complex gene mutations. The most reliable means of differentiating a perithyroidal paragangliomas from other neuroendocrine tumors of this region, including medullary thyroid carcinoma and parathyroid neoplasms, is performing cytokeratin; paragangliomas are a cytokeratin-negative neuroendocrine tumor. They also are helpfully positive for GATA3. Loss of staining for SDHB protein correlates with SDH complex gene mutations and is frequently used as a prognostic marker.
Incorrect
Answer: PARAGANGLIOMA
Histology: The tumor is actually centered outside the thyroid gland. It consists of a nested proliferation of cells with abundant amphophilic cytoplasm embedded in a variably vascular and fibrotic stroma. Nuclei show moderate variation in size with speckled chromatin and occasional prominent nucleoli. Tumor cells are positive for synaptophysin, INSM1, and GATA3, and negative for AE1/AE3 with retained SDHB. Sustentacular cells are positive for S100.
Discussion: In the head and neck, paragangliomas most commonly arise in the carotid body but can affect a wide range of sites, including within or adjacent to the thyroid gland. Because these tumors frequently arise from parasympathetic paraganglia, they are most often non-functional and generally present with mass effect. Historically, tumor behavior has been regarded as unpredictable, without good correlation to histologic features. However, most malignant paragangliomas have recently been associated with SDH complex gene mutations. The most reliable means of differentiating a perithyroidal paragangliomas from other neuroendocrine tumors of this region, including medullary thyroid carcinoma and parathyroid neoplasms, is performing cytokeratin; paragangliomas are a cytokeratin-negative neuroendocrine tumor. They also are helpfully positive for GATA3. Loss of staining for SDHB protein correlates with SDH complex gene mutations and is frequently used as a prognostic marker.
Presented by Dr. Lisa Rooper and prepared by Dr. Tatianna Larman.
A 55 year old man with hoarseness, found to have a laryngeal mass.
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A 55 year old man with hoarseness, found to have a laryngeal mass. Choose the correct diagnosis.
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Answer: HYBRID CARCINOMA
Histology: The laryngeal mass consists of a exophytic squamous proliferation with prominent finger-like projections and abundant keratin production. Although superficial tumor cells demonstrate bland cytology, deeper areas demonstrate prominent more marked nuclear pleomorphism and disordered maturation. Additionally, at the base of the lesion, there are irregular nests of atypical squamous cells showing downward extension into the underlying stroma.
Discussion: The spectrum of verrucous lesions in the larynx can present a difficult differential diagnosis, especially on superficial biopsies. Both verrucous hyperplasia and verrucous carcinoma should display uniformly bland cytology. While verrucous hyperplasia should not extend deeper than the surrounding normal epithelium, verrucous carcinoma will demonstrate pushing growth into the stroma. However, verrucous carcinoma should not demonstrate overtly infiltrative growth. Hybrid carcinoma has areas that are identical to verrucous carcinoma but harbors other areas of more conventional squamous cell carcinoma with cytologic atypia and frank invasion. It is essential to distinguish these lesions because verrucous carcinomas lack metastatic potential while hybrid carcinomas behave like conventional squamous cell carcinoma.
Incorrect
Answer: HYBRID CARCINOMA
Histology: The laryngeal mass consists of a exophytic squamous proliferation with prominent finger-like projections and abundant keratin production. Although superficial tumor cells demonstrate bland cytology, deeper areas demonstrate prominent more marked nuclear pleomorphism and disordered maturation. Additionally, at the base of the lesion, there are irregular nests of atypical squamous cells showing downward extension into the underlying stroma.
Discussion: The spectrum of verrucous lesions in the larynx can present a difficult differential diagnosis, especially on superficial biopsies. Both verrucous hyperplasia and verrucous carcinoma should display uniformly bland cytology. While verrucous hyperplasia should not extend deeper than the surrounding normal epithelium, verrucous carcinoma will demonstrate pushing growth into the stroma. However, verrucous carcinoma should not demonstrate overtly infiltrative growth. Hybrid carcinoma has areas that are identical to verrucous carcinoma but harbors other areas of more conventional squamous cell carcinoma with cytologic atypia and frank invasion. It is essential to distinguish these lesions because verrucous carcinomas lack metastatic potential while hybrid carcinomas behave like conventional squamous cell carcinoma.
Presented by Dr. Elizabeth Thompson and prepared by Dr. Tatianna Larman.
An intradural mass in a 40 year old woman.
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An intradural mass in a 40 year old woman. Choose the correct diagnosis.
Correct
Answer: Schwannoma
Histology: Sections of the lesion show elongated spindled cells with a “wavy” shape and tapered ends with abundant intervening collagen. Cellularity varies throughout and there is prominent nuclear palisading. There are vessels of varying sizes, often with hyalinized walls, and focal areas of cystic change.
Discussion: This is a beautiful example of a schwannoma with classic histologic features of Antoni A ares (cellular, monomorphic spindled Schwann cells in a variably collagenous stroma) and Antoni B areas (hypocelluar, spindled cells in myxoid matrix). Antoni A areas often show nuclear palisading and parallel arrays with intervening eosinophilic cytoplasm known as Verocay bodies (very prominent in this example). Degenerative or so-called “ancient change” in schwannomas is common and includes hyalinization of blood vessels, stromal hemorrhage, cystic change and calcification. Variants include: ancient schwannoma, cellular schwannoma (up to 10 mitoses per 10 hpf, can see focal necrosis and, inconspicuous or focal Antoni B areas), plexiform schwannoma, melanotic schwannoma, epithelioid schwannoma, and glandular schwannoma. Schwannomas are strongly and diffusely S100 positive. Those in the GI tract are also often GFAP positive and those in the retroperitoneum and mediastinum can be keratin positive. The vast majority have a benign course with only rare recurrence. Malignant transformation is very rare. They are typically solitary lesions. Bilateral schwannomas of the acoustic nerve are an almost pathognomonic feature of NF2. Multiple peripheral schwannomas (schwannomatosis) can develop in patients with SMARCB1 (INI1) mutations as well as NF2 inactivation, and can be familial.
Incorrect
Answer: Schwannoma
Histology: Sections of the lesion show elongated spindled cells with a “wavy” shape and tapered ends with abundant intervening collagen. Cellularity varies throughout and there is prominent nuclear palisading. There are vessels of varying sizes, often with hyalinized walls, and focal areas of cystic change.
Discussion: This is a beautiful example of a schwannoma with classic histologic features of Antoni A ares (cellular, monomorphic spindled Schwann cells in a variably collagenous stroma) and Antoni B areas (hypocelluar, spindled cells in myxoid matrix). Antoni A areas often show nuclear palisading and parallel arrays with intervening eosinophilic cytoplasm known as Verocay bodies (very prominent in this example). Degenerative or so-called “ancient change” in schwannomas is common and includes hyalinization of blood vessels, stromal hemorrhage, cystic change and calcification. Variants include: ancient schwannoma, cellular schwannoma (up to 10 mitoses per 10 hpf, can see focal necrosis and, inconspicuous or focal Antoni B areas), plexiform schwannoma, melanotic schwannoma, epithelioid schwannoma, and glandular schwannoma. Schwannomas are strongly and diffusely S100 positive. Those in the GI tract are also often GFAP positive and those in the retroperitoneum and mediastinum can be keratin positive. The vast majority have a benign course with only rare recurrence. Malignant transformation is very rare. They are typically solitary lesions. Bilateral schwannomas of the acoustic nerve are an almost pathognomonic feature of NF2. Multiple peripheral schwannomas (schwannomatosis) can develop in patients with SMARCB1 (INI1) mutations as well as NF2 inactivation, and can be familial.
Presented by Dr. Elizabeth Thompson and prepared by Dr. Tatianna Larman.
A sinonasal mass in a 55 year old woman.
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A sinonasal mass in a 55 year old. Choose the correct diagnosis.
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Answer: Adenoid cystic carcinoma
Histology: This tumor is comprised of a combination of patterns: cribriform, tubular and solid, formed primarily by dark, basaloid round-to-angulated cells. A second population of round cells with eosinophilic cytoplasm can also be appreciated, imparting a biphasic appearance to certain areas. Matrix material can be seen in some of the “punched out” spaces in the cribriform areas. Significant cellular pleomorphism and mitotic activity are not appreciated.
Discussion: This tumor is comprised of a combination of patterns: cribriform, tubular and solid, formed primarily by dark, basaloid round-to-angulated cells. A second population of round cells with eosinophilic cytoplasm can also be appreciated, imparting a biphasic appearance to certain areas. Matrix material can be seen in some of the “punched out” spaces in the cribriform areas. Significant cellular pleomorphism and mitotic activity are not appreciated.
Incorrect
Answer: Adenoid cystic carcinoma
Histology: This tumor is comprised of a combination of patterns: cribriform, tubular and solid, formed primarily by dark, basaloid round-to-angulated cells. A second population of round cells with eosinophilic cytoplasm can also be appreciated, imparting a biphasic appearance to certain areas. Matrix material can be seen in some of the “punched out” spaces in the cribriform areas. Significant cellular pleomorphism and mitotic activity are not appreciated.
Discussion: This tumor is comprised of a combination of patterns: cribriform, tubular and solid, formed primarily by dark, basaloid round-to-angulated cells. A second population of round cells with eosinophilic cytoplasm can also be appreciated, imparting a biphasic appearance to certain areas. Matrix material can be seen in some of the “punched out” spaces in the cribriform areas. Significant cellular pleomorphism and mitotic activity are not appreciated.
Presented by Dr. Andres Matoso and prepared by Dr. Tatianna Larman.
A young adult with a paratesticular tumor.
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A young adult with a paratesticular mass. Choose the correct diagnosis.
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Answer: Rhabdomyosarcoma
Histology and discussion: This tumor is composed of a monomorphic population of round and spindle cells with high nuclear to cytoplasmic ratio and scant cytoplasm. Nucleoli are inconspicuous. There is brisk mitotic activity and frequent apoptotic bodies. Given the location and the morphologic features, rhabdomyosarcoma is at the top of the differential. Diffuse nuclear immunostaining for myogenin D confirms the diagnosis, as it is a very specific marker. Rhabdomyosarcomas are also frequently positive for desmin, however this marker is not helpful in differentiating it from leiomyosarcoma. Among the subtypes of rhabdomyosarcoma, this is consistent with embryonal given its primitive poorly differentiated morphology. Cytogenetically, embryonal rhabdomyosarcoma is characterized by LOH of chromosome 11p15.5, trisomy 8, and upregulation of GLI1 and PTCH1. Embryonal rhabdomyosarcoma has an intermediate prognosis.
References:
1. Int J Surg Pathol. 2017 Apr 1:1066896917707040.
2. Pediatr Blood Cancer. 2017 Feb 16. doi: 10.1002/pbc.26486.
Incorrect
Answer: Rhabdomyosarcoma
Histology and discussion: This tumor is composed of a monomorphic population of round and spindle cells with high nuclear to cytoplasmic ratio and scant cytoplasm. Nucleoli are inconspicuous. There is brisk mitotic activity and frequent apoptotic bodies. Given the location and the morphologic features, rhabdomyosarcoma is at the top of the differential. Diffuse nuclear immunostaining for myogenin D confirms the diagnosis, as it is a very specific marker. Rhabdomyosarcomas are also frequently positive for desmin, however this marker is not helpful in differentiating it from leiomyosarcoma. Among the subtypes of rhabdomyosarcoma, this is consistent with embryonal given its primitive poorly differentiated morphology. Cytogenetically, embryonal rhabdomyosarcoma is characterized by LOH of chromosome 11p15.5, trisomy 8, and upregulation of GLI1 and PTCH1. Embryonal rhabdomyosarcoma has an intermediate prognosis.
References:
1. Int J Surg Pathol. 2017 Apr 1:1066896917707040.
2. Pediatr Blood Cancer. 2017 Feb 16. doi: 10.1002/pbc.26486.
Presented by Dr. Andres Matoso and prepared by Dr. Tatianna Larman.
An adult with a renal mass.
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An adult with a renal mass. Choose the correct diagnosis.
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Answer: Multilocular cystic renal cell neoplasm of low malignant potential
Histology and discussion: Multilocular cystic renal cell neoplasm of low malignant potential is composed of a multiple cystic spaces lined by clear cells with low nuclear grade (ISUP nuclear grade 1 or 2). The stroma may have some clusters of clear cells but they are not expanding or forming solid nodules. The specimen has to be sample generously (at least one cassette per cm with 2 or 3 sections per cassette) with especial attention to sample well any potential solid areas. If there is any solid growth, then clear cell renal cell carcinoma with cystic change should be entertained as the diagnosis. In contrast, if the stroma shows no cluster of clear cells, then atypical renal cyst should be considered. The prognosis is excellent with a 5 year survival rate of 100% in the small series published.
Answer: Multilocular cystic renal cell neoplasm of low malignant potential
Histology and discussion: Multilocular cystic renal cell neoplasm of low malignant potential is composed of a multiple cystic spaces lined by clear cells with low nuclear grade (ISUP nuclear grade 1 or 2). The stroma may have some clusters of clear cells but they are not expanding or forming solid nodules. The specimen has to be sample generously (at least one cassette per cm with 2 or 3 sections per cassette) with especial attention to sample well any potential solid areas. If there is any solid growth, then clear cell renal cell carcinoma with cystic change should be entertained as the diagnosis. In contrast, if the stroma shows no cluster of clear cells, then atypical renal cyst should be considered. The prognosis is excellent with a 5 year survival rate of 100% in the small series published.
Histology and discussion: SDH deficient GISTs occur almost exclusively in the stomach. Recently, a duodenal one was reported in a patient with SDHB germline mutation. They are characterized by epithelioid morphology, plexiform (multinodular) architecture and metastasis to lymph nodes. They may be part of the Carney-Stratakis syndrome (paraganglioma and GIST) or Carney’s triad (paraganglioma, GIST and pulmonary chondroma). These tumors lack kinase mutations but they can still be CD117 and DOG1 positive by immunohistochemistry. SDHB is lost by immunohistochemistry, which is diagnostic of SDH-deficient GIST. Negative immunostain for SDHB indicates either germline mutation in any of the SDH genes or SDHC hypermethylation. Rare somatic mutations have been reported. Conventional risk stratification does not accurately predict prognosis in this type of GISTs.
References:
1. Am J Surg Pathol. 2016 Dec;40(12):1616-1621.
2. J Clin Endocrinol Metab. 2017 May 1;102(5):1447-1450.
3. Endocrine. 2014 Nov;47(2):401-8.
Histology and discussion: SDH deficient GISTs occur almost exclusively in the stomach. Recently, a duodenal one was reported in a patient with SDHB germline mutation. They are characterized by epithelioid morphology, plexiform (multinodular) architecture and metastasis to lymph nodes. They may be part of the Carney-Stratakis syndrome (paraganglioma and GIST) or Carney’s triad (paraganglioma, GIST and pulmonary chondroma). These tumors lack kinase mutations but they can still be CD117 and DOG1 positive by immunohistochemistry. SDHB is lost by immunohistochemistry, which is diagnostic of SDH-deficient GIST. Negative immunostain for SDHB indicates either germline mutation in any of the SDH genes or SDHC hypermethylation. Rare somatic mutations have been reported. Conventional risk stratification does not accurately predict prognosis in this type of GISTs.
References:
1. Am J Surg Pathol. 2016 Dec;40(12):1616-1621.
2. J Clin Endocrinol Metab. 2017 May 1;102(5):1447-1450.
3. Endocrine. 2014 Nov;47(2):401-8.
Question 2 of 2
2. Question
An adult with a renal mass. Choose the correct diagnosis.
Correct
Answer: Multilocular cystic renal cell neoplasm of low malignant potential
Histology and discussion: Multilocular cystic renal cell neoplasm of low malignant potential is composed of a multiple cystic spaces lined by clear cells with low nuclear grade (ISUP nuclear grade 1 or 2). The stroma may have some clusters of clear cells but they are not expanding or forming solid nodules. The specimen has to be sample generously (at least one cassette per cm with 2 or 3 sections per cassette) with especial attention to sample well any potential solid areas. If there is any solid growth, then clear cell renal cell carcinoma with cystic change should be entertained as the diagnosis. In contrast, if the stroma shows no cluster of clear cells, then atypical renal cyst should be considered. The prognosis is excellent with a 5 year survival rate of 100% in the small series published.
Answer: Multilocular cystic renal cell neoplasm of low malignant potential
Histology and discussion: Multilocular cystic renal cell neoplasm of low malignant potential is composed of a multiple cystic spaces lined by clear cells with low nuclear grade (ISUP nuclear grade 1 or 2). The stroma may have some clusters of clear cells but they are not expanding or forming solid nodules. The specimen has to be sample generously (at least one cassette per cm with 2 or 3 sections per cassette) with especial attention to sample well any potential solid areas. If there is any solid growth, then clear cell renal cell carcinoma with cystic change should be entertained as the diagnosis. In contrast, if the stroma shows no cluster of clear cells, then atypical renal cyst should be considered. The prognosis is excellent with a 5 year survival rate of 100% in the small series published.
Presented by Dr. Pedram Argani and prepared by Dr. Tatianna Larman.
A 52 year old woman with a renal mass.
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A 52 year old woman with a renal mass. Choose the correct diagnosis.
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Answer: Angiomyolipoma with epithelial cysts
Histology: This is a predominantly stromal lesion which has entrapped renal tubules that project beyond capsule of the kidney. The stromal component is typical of a stromal predominant angiomyolipoma, in that it features irregular dysplastic blood vessels associated with plump spindle cells with pale to eosinophilic cytoplasm that form short fascicles. Within the lesion is entrapped renal tubular epithelium which is bland cytologically, though it is associated with a cellular, more primitive appearing subepithelial stroma that resembles endometrium. The angiomyolipoma component labels for HMB45 and Melan A, whereas the tubular epithelium labels for PAX8, consistent with entrapped renal tubules. There is accentuation of estrogen receptor labeling in the subepithelial stromal, which supports Müllerian differentiation. These are the typical features of angiomyolipoma with epithelial cysts (AMLEC).
Discussion: Synovial sarcomas can be extensively cystic in the kidney, and associated similarly with entrapped native renal tubules. However, synovial sarcomas are composed of more primitive appearing basophilic spindle cells, associated with frequent mitotic activity. Synovial sarcoma would not label for melanocytic markers like AMLEC. Mixed epithelial stromal tumor often contains smooth muscle stroma associated with dilated epithelial cysts. However, the stroma is usually that of fibrous tissue or typical smooth muscle, and does not label for the melanocytic marker HMB45. Endometriosis would not be associated with dysplastic blood vessels, and the aberrant smooth muscle that is typical of angiomyolipoma.
Reference(s):
– American Journal of Surgical Pathology 2006; 30:593-9
Incorrect
Answer: Angiomyolipoma with epithelial cysts
Histology: This is a predominantly stromal lesion which has entrapped renal tubules that project beyond capsule of the kidney. The stromal component is typical of a stromal predominant angiomyolipoma, in that it features irregular dysplastic blood vessels associated with plump spindle cells with pale to eosinophilic cytoplasm that form short fascicles. Within the lesion is entrapped renal tubular epithelium which is bland cytologically, though it is associated with a cellular, more primitive appearing subepithelial stroma that resembles endometrium. The angiomyolipoma component labels for HMB45 and Melan A, whereas the tubular epithelium labels for PAX8, consistent with entrapped renal tubules. There is accentuation of estrogen receptor labeling in the subepithelial stromal, which supports Müllerian differentiation. These are the typical features of angiomyolipoma with epithelial cysts (AMLEC).
Discussion: Synovial sarcomas can be extensively cystic in the kidney, and associated similarly with entrapped native renal tubules. However, synovial sarcomas are composed of more primitive appearing basophilic spindle cells, associated with frequent mitotic activity. Synovial sarcoma would not label for melanocytic markers like AMLEC. Mixed epithelial stromal tumor often contains smooth muscle stroma associated with dilated epithelial cysts. However, the stroma is usually that of fibrous tissue or typical smooth muscle, and does not label for the melanocytic marker HMB45. Endometriosis would not be associated with dysplastic blood vessels, and the aberrant smooth muscle that is typical of angiomyolipoma.
Reference(s):
– American Journal of Surgical Pathology 2006; 30:593-9
Presented by Dr. Pedram Argani and prepared by Dr. Tatianna Larman.
A 72 year old woman with a renal mass.
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A 72 year old woman with a renal mass. Choose the correct diagnosis.
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Answer: Hybrid oncocytoma-chromophobe tumor
Histology: The majority of this lesion, particularly the central portions, has the typical features of oncocytoma; specifically, nests of oncocytic cells in edematous stroma, with the cells having rounded nuclei. However, at the edge of the lesion, the architecture is more solid and the nuclei are more irregular, with frequent binucleate forms associated with perinuclear halos. These are the cytologic features that are more in keeping with chromophobe carcinoma. Hence, the best diagnosis for this lesion is a hybrid oncocytoma-chromophobe tumor. These lesions have had a benign course in clinical follow-up, though they are associated with Birt-Hogg-Dubé syndrome.
Discussion: The mixed morphology precludes the diagnosis of either oncocytoma or chromophobe carcinoma. SDH-deficient renal cell carcinomas typically show cytoplasmic vacuoles along with cytoplasmic inclusions which correspond to giant mitochondria, and show loss of SDHB immunoreactivity.
Incorrect
Answer: Hybrid oncocytoma-chromophobe tumor
Histology: The majority of this lesion, particularly the central portions, has the typical features of oncocytoma; specifically, nests of oncocytic cells in edematous stroma, with the cells having rounded nuclei. However, at the edge of the lesion, the architecture is more solid and the nuclei are more irregular, with frequent binucleate forms associated with perinuclear halos. These are the cytologic features that are more in keeping with chromophobe carcinoma. Hence, the best diagnosis for this lesion is a hybrid oncocytoma-chromophobe tumor. These lesions have had a benign course in clinical follow-up, though they are associated with Birt-Hogg-Dubé syndrome.
Discussion: The mixed morphology precludes the diagnosis of either oncocytoma or chromophobe carcinoma. SDH-deficient renal cell carcinomas typically show cytoplasmic vacuoles along with cytoplasmic inclusions which correspond to giant mitochondria, and show loss of SDHB immunoreactivity.
Presented by Dr. Pedram Argani and prepared by Dr. Tatianna Larman.
A 50 year old woman with a tongue mass.
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A 50 year old woman with a tongue mass. Choose the correct diagnosis.
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Answer: Granular cell tumor
Histology: The striking appearance at low power of this slide is that of irregular nests of squamous epithelium appear too permeate deeply between skeletal muscle bundles. On closer inspection, the nests of squamous epithelium show minimal cytological atypia. Importantly, one can appreciate surrounding these nests a population of epithelioid cells with granular cytoplasm. In some areas the granules are large and form small inclusions. These are the typical features of a granular cell tumor, which characteristically incites pseudoepitheliomatous squamous hyperplasia. The granular cell tumor in this case labeled for S100 and inhibin, supporting the diagnosis.
Discussion: Granular cell tumor with associated pseudoepitheliomatous squamous hyperplasia is a known mimic of squamous cell carcinoma. The absence of cytologic atypia as well as attention to the stroma helps distinguish this from invasive squamous cell carcinoma. The absence of atypia also argues against carcinoma in situ, as does the irregular nature of some of the nests on the slide. Of note, there are essentially no salivary gland type ducts in this section. Median rhomboid glossitis is associated with Candida infection. One can see rounded nests of squamous epithelium which extend down from the surface, but not the florid irregular small nests seen in the current case. One should also appreciate Candida superficially in the histologic sections as the etiology.
One should always pay attention to the stroma before rendering a diagnosis of invasive squamous cell carcinoma.
Incorrect
Answer: Granular cell tumor
Histology: The striking appearance at low power of this slide is that of irregular nests of squamous epithelium appear too permeate deeply between skeletal muscle bundles. On closer inspection, the nests of squamous epithelium show minimal cytological atypia. Importantly, one can appreciate surrounding these nests a population of epithelioid cells with granular cytoplasm. In some areas the granules are large and form small inclusions. These are the typical features of a granular cell tumor, which characteristically incites pseudoepitheliomatous squamous hyperplasia. The granular cell tumor in this case labeled for S100 and inhibin, supporting the diagnosis.
Discussion: Granular cell tumor with associated pseudoepitheliomatous squamous hyperplasia is a known mimic of squamous cell carcinoma. The absence of cytologic atypia as well as attention to the stroma helps distinguish this from invasive squamous cell carcinoma. The absence of atypia also argues against carcinoma in situ, as does the irregular nature of some of the nests on the slide. Of note, there are essentially no salivary gland type ducts in this section. Median rhomboid glossitis is associated with Candida infection. One can see rounded nests of squamous epithelium which extend down from the surface, but not the florid irregular small nests seen in the current case. One should also appreciate Candida superficially in the histologic sections as the etiology.
One should always pay attention to the stroma before rendering a diagnosis of invasive squamous cell carcinoma.
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