Presented by Dr. Argani and prepared by Austin McCuiston.
This is a 53 year old male with an abdominal wall sinus tract/chronic hematoma status post remote panniculectomy.
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This is a 53 year old male with an abdominal wall sinus tract/chronic hematoma status post remote panniculectomy.
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Answer: Angiosarcoma
Histologic Description: There are areas of organizing hematoma here; however; there are also highly pleomorphic cells with prominent nucleoli that form vascular spaces. Mitotic figures are frequent and pericytes are not noted. The neoplastic cells diffusely label for CD34, supporting the diagnosis.
Differential Diagnosis: Papillary endothelial hyperplasia may demonstrate an anastomosing vascular pattern; however, this should be associated with fibrin and lack cytologic atypia. Granulation tissue would have bland capillaries and lack the atypia of the current case. Kaposi’s sarcoma would have blander nuclei that typically are spindled, and would be associated with prominent inflammatory component. Angiosarcoma may be associated with foreign material, such as AV-fistulas. In the current case, this along with chronic lymphedema associated with the fistula tract may have predisposed to this malignancy.
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Answer: Angiosarcoma
Histologic Description: There are areas of organizing hematoma here; however; there are also highly pleomorphic cells with prominent nucleoli that form vascular spaces. Mitotic figures are frequent and pericytes are not noted. The neoplastic cells diffusely label for CD34, supporting the diagnosis.
Differential Diagnosis: Papillary endothelial hyperplasia may demonstrate an anastomosing vascular pattern; however, this should be associated with fibrin and lack cytologic atypia. Granulation tissue would have bland capillaries and lack the atypia of the current case. Kaposi’s sarcoma would have blander nuclei that typically are spindled, and would be associated with prominent inflammatory component. Angiosarcoma may be associated with foreign material, such as AV-fistulas. In the current case, this along with chronic lymphedema associated with the fistula tract may have predisposed to this malignancy.
Presented by Dr. Argani and prepared by Austin McCuiston.
This is 42 year old female with a breast capsule mass.
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This is 42 year old female with a breast capsule mass.
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Answer: Capsular anaplastic large cell lymphoma
Histologic Description: Within the fibrin surrounding the capsule of the implant are large pleomorphic cells associated with mitotic activity. These cells have irregular nuclear membranes, and are discohesive. These cells labeled for CD30 and CD43 along with EMA.
Differential Diagnosis: The malignant cells of capsular anaplastic large cell lymphoma may demonstrate some cohesion and this along with the EMA immunoreactivity may suggest a poorly differentiated carcinoma, which would be a more common diagnosis. The irregular nuclear membranes along with the CD30 immunoreactivity supports the diagnosis of lymphoma. Angiosarcoma would be vasoformative, and would label for CD34 but not CD30. The atypia seen in the current case is much greater than that seen in reactive changes.
Cases of breast implant associated anaplastic large cell lymphoma that present as a seroma (effusion only) have a favorable prognosis following surgery, whereas those that present with a mass extending beyond the fibrous capsule have a worse prognosis and may require chemotherapy.
Reference: Archives of Pathology and Laboratory Medicine 2014; 138:842-846.
Incorrect
Answer: Capsular anaplastic large cell lymphoma
Histologic Description: Within the fibrin surrounding the capsule of the implant are large pleomorphic cells associated with mitotic activity. These cells have irregular nuclear membranes, and are discohesive. These cells labeled for CD30 and CD43 along with EMA.
Differential Diagnosis: The malignant cells of capsular anaplastic large cell lymphoma may demonstrate some cohesion and this along with the EMA immunoreactivity may suggest a poorly differentiated carcinoma, which would be a more common diagnosis. The irregular nuclear membranes along with the CD30 immunoreactivity supports the diagnosis of lymphoma. Angiosarcoma would be vasoformative, and would label for CD34 but not CD30. The atypia seen in the current case is much greater than that seen in reactive changes.
Cases of breast implant associated anaplastic large cell lymphoma that present as a seroma (effusion only) have a favorable prognosis following surgery, whereas those that present with a mass extending beyond the fibrous capsule have a worse prognosis and may require chemotherapy.
Reference: Archives of Pathology and Laboratory Medicine 2014; 138:842-846.
Presented by Dr. Argani and prepared by Austin McCuiston.
This is a 70 year old male with unexpected colonic perforation following laparotomy.
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This is a 70 year old male with unexpected colonic perforation following laparotomy.
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Answer: Collagenous colitis
Histologic Description: While the area of perforation demonstrated prominent granulation tissue, the surrounding bowel demonstrates increased lamia propria plasma cells, along with increased intraepithelial lymphocytes with epithelial damage. Close inspection reveals that the subepithelial basement membrane is thickened, and has an irregular border with the underlying lamina propria. These are the typical features of collagenous colitis.
Differential Diagnosis: Ulcerative colitis would be associated with more prominent crypt distortion and frequently active colitis. Infectious colitis would typically have an active colitis pattern, and would lack the prominent intraepithelial lymphocytes of the current case along with thickened basement membrane. Lymphocytic colitis is very similar to collagenous colitis in that both are microscopic findings seen in patients with watery diarrhea whose colonoscopies are grossly normal, but it is distinguished by the absence of a thickened subepithelial basement membrane.
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Answer: Collagenous colitis
Histologic Description: While the area of perforation demonstrated prominent granulation tissue, the surrounding bowel demonstrates increased lamia propria plasma cells, along with increased intraepithelial lymphocytes with epithelial damage. Close inspection reveals that the subepithelial basement membrane is thickened, and has an irregular border with the underlying lamina propria. These are the typical features of collagenous colitis.
Differential Diagnosis: Ulcerative colitis would be associated with more prominent crypt distortion and frequently active colitis. Infectious colitis would typically have an active colitis pattern, and would lack the prominent intraepithelial lymphocytes of the current case along with thickened basement membrane. Lymphocytic colitis is very similar to collagenous colitis in that both are microscopic findings seen in patients with watery diarrhea whose colonoscopies are grossly normal, but it is distinguished by the absence of a thickened subepithelial basement membrane.
Presented by Dr. Cimino-Mathews and prepared by Austin McCuiston.
20 year-old male with a pancreatic mass.
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20 year-old male with a pancreatic mass.
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Diagnosis: Solid Pseudopapillary neoplasm
Histology: This pancreatic resection specimen displays a large mass with a fibrotic rim with adjacent histologically unremarkable pancreatic parenchyma. The lesion is highly vascular with areas of hemorrhage, extravasated red blood cells, cystic degeneration, fibrin, collections of histiocytes, and cholesterol clefts. The lesional cells are loosely cohesive with papillary fronds, but in areas form solid nests. The nuclei are predominantly round, with evenly dispersed chromatin. The cytoplasm is amphophilic to eosinophilic, and occasional intracytoplasmic hyaline globules can be identified. Scattered mitotic figures are readily apparent, and regions of tumoral necrosis are present. The lesion is immunoreactive for nuclear beta-catenin, and is negative for synaptophysin, chromogranin, BCL10, and trypsin.
Discussion: This is a solid pseudopapillary neoplasm of the pancreas, which classically occurs in the pancreatic tail of young adult women. However, these lesions can occur in children as well as older adults. These lesions typically have an indolent course and can be cured with complete resection; however, they do have low malignant potential and can metastasize, for example, to the liver. Solid pseudopapillary neoplasms are in the differential diagnosis of solid cellular neoplasms of the pancreas, which include pancreatic neuroendocrine tumor (PanNET), microcystic/solid variant of serous cystadenoma, acinar cell carcinoma, and metastases to the pancreas. The classic morphologic features of solid pseudopapillary neoplasm are in the name…the lesion is variably solid and in areas (pseudo)papillary! The lesion appears to have papillary fronds due to the loosely cohesive nature of the cells. The presence of foamy macrophages and cholesterol clefts also favor the diagnosis. The nuclei may be oval with coffee-bean grooves, however, as seen in this case, can also be oval with evenly dispersed chromatin (mimicking a PanNET). The presence of intracytoplasmic hyaline globules can be seen in either PanNET or solid pseudopapillary neoplasm. The differential diagnosis can be resolved with immunohistochemistry. The most useful immunostain for solid pseudopapilary neoplasms is beta-catenin, which will show nuclear labeling; variable positive labeling can also be seen with CD10, cyclin D1, AR, PR, CD56, synaptophysin and cytokeratin. PanNETs label with neuroendocrine markers and cytokeratin, and acinar cell carcinomas label with BCL10 and trypsin, but neither will show nuclear beta-catenin labeling.
References:
1. Abraham SC, Klimstra DS, Wilentz RE, Yeo CJ, Conlon K, Brennan M, Cameron JL, Wu TT, Hruban RH. Solid-pseudopapillary tumors of the pancreas are genetically distinct from pancreatic ductal adenocarcinomas and almost always harbor beta-catenin mutations. Am J Pathol. 2002 Apr;160(4):1361-9.
2. Meriden Z, Shi C, Edil BH, Ellison T, Wolfgang CL, Cornish TC, Schulick RD, Hruban RH. Hyaline globules in neuroendocrine and solid-pseudopapillary neoplasms of the pancreas: a clue to the diagnosis. Am J Surg Pathol. 2011 Jul;35(7):981-8
3. Law JK, Ahmed A, Singh VK […] Hruban RH, Wolfgang CL, Lennon AM. A systematic review of solid-pseudopapillary neoplasms: are these rare lesions? Pancreas. 2014 Apr;43(3):331-7.
Incorrect
Diagnosis: Solid Pseudopapillary neoplasm
Histology: This pancreatic resection specimen displays a large mass with a fibrotic rim with adjacent histologically unremarkable pancreatic parenchyma. The lesion is highly vascular with areas of hemorrhage, extravasated red blood cells, cystic degeneration, fibrin, collections of histiocytes, and cholesterol clefts. The lesional cells are loosely cohesive with papillary fronds, but in areas form solid nests. The nuclei are predominantly round, with evenly dispersed chromatin. The cytoplasm is amphophilic to eosinophilic, and occasional intracytoplasmic hyaline globules can be identified. Scattered mitotic figures are readily apparent, and regions of tumoral necrosis are present. The lesion is immunoreactive for nuclear beta-catenin, and is negative for synaptophysin, chromogranin, BCL10, and trypsin.
Discussion: This is a solid pseudopapillary neoplasm of the pancreas, which classically occurs in the pancreatic tail of young adult women. However, these lesions can occur in children as well as older adults. These lesions typically have an indolent course and can be cured with complete resection; however, they do have low malignant potential and can metastasize, for example, to the liver. Solid pseudopapillary neoplasms are in the differential diagnosis of solid cellular neoplasms of the pancreas, which include pancreatic neuroendocrine tumor (PanNET), microcystic/solid variant of serous cystadenoma, acinar cell carcinoma, and metastases to the pancreas. The classic morphologic features of solid pseudopapillary neoplasm are in the name…the lesion is variably solid and in areas (pseudo)papillary! The lesion appears to have papillary fronds due to the loosely cohesive nature of the cells. The presence of foamy macrophages and cholesterol clefts also favor the diagnosis. The nuclei may be oval with coffee-bean grooves, however, as seen in this case, can also be oval with evenly dispersed chromatin (mimicking a PanNET). The presence of intracytoplasmic hyaline globules can be seen in either PanNET or solid pseudopapillary neoplasm. The differential diagnosis can be resolved with immunohistochemistry. The most useful immunostain for solid pseudopapilary neoplasms is beta-catenin, which will show nuclear labeling; variable positive labeling can also be seen with CD10, cyclin D1, AR, PR, CD56, synaptophysin and cytokeratin. PanNETs label with neuroendocrine markers and cytokeratin, and acinar cell carcinomas label with BCL10 and trypsin, but neither will show nuclear beta-catenin labeling.
References:
1. Abraham SC, Klimstra DS, Wilentz RE, Yeo CJ, Conlon K, Brennan M, Cameron JL, Wu TT, Hruban RH. Solid-pseudopapillary tumors of the pancreas are genetically distinct from pancreatic ductal adenocarcinomas and almost always harbor beta-catenin mutations. Am J Pathol. 2002 Apr;160(4):1361-9.
2. Meriden Z, Shi C, Edil BH, Ellison T, Wolfgang CL, Cornish TC, Schulick RD, Hruban RH. Hyaline globules in neuroendocrine and solid-pseudopapillary neoplasms of the pancreas: a clue to the diagnosis. Am J Surg Pathol. 2011 Jul;35(7):981-8
3. Law JK, Ahmed A, Singh VK […] Hruban RH, Wolfgang CL, Lennon AM. A systematic review of solid-pseudopapillary neoplasms: are these rare lesions? Pancreas. 2014 Apr;43(3):331-7.
Histology: This pancreatic resection specimen displays areas of unremarkable pancreatic parenchyma, with separate regions displaying chronic pancreatitis, suggestive of ductal obstruction. Within the pancreas is a fibrotic tumor characterized by cells arranged in nests, trabeculae, single file, and single cell patterns. The cells have amphophilic granular cytoplasm and oval to rounded nuclei with finely dispersed “salt and pepper chromatin,” occasionally prominent nucleoli, and scattered degenerative-type atypia. Mitotic figures are inconspicuous. Perinerual invasion is readily identified, and metastatic foci are present within a peripancreatic lymph node. Immunotains show the lesional cells to be immunoreactive for synaptophysin and serotonin, and negative for BCL10, with a Ki67 proliferation index of <2%.
Discussion: This lesion exhibits the morphologic and immunophenotypic features of a well-differentiated, pancreatic neuroendocrine tumor (PanNET), which are further subdivided on the basis of tumor grade and (to an extent) tumor functionality. PanNETs are graded on the basis of the mitotic count per high power field (HPF) or the Ki67 proliferation index (measured as a percentage of cells with nuclear Ki67 labeling), and the higher of the two measures is used to determine the grade. Well-differentiated PanNETs display bland morphologic features (i.e., lack necrosis, lack features of small cell or large cell neuroendocrine carcinoma) and are classified as Grade 1 (mitoses <2/10 HPF or Ki67 <2%), Grade 2 (mitoses 2-20/10 HPF or Ki67 3-20%), or Grade 3 (mitoses >20/10 HPF or Ki67 >20%). Poorly differentiated pancreatic neuroendocrine carcinoma is also Grade 3 (mitoses >20/10 HPF or Ki67 >20%) but in addition displays histologic features of small cell carcinoma or large cell neuroendocrine carcinoma. The differential diagnosis of a solid cellular neoplasm in the pancreas includes acinar cell carcinoma, solid pseudopapillary neoplasms, metastases to the pancreas, and possibly ductal adenocarcinoma. In this particular tumor, there is abundant fibrosis, which can mimic the desmoplasia seen in ductal adenocarcinoma. However, the presence of the fibrosis as well as the trabecular growth pattern is a clue to the functional status of this tumor: it is a serotonin-secreting, well-differentiated grade 1 PanNET. Serotonin-secreting PanNETs characteristically show fibrosis, calcification, large duct involvement, and trabecular growth. Not all PanNETs are functional, but all should display some degree of labeling for neuroendocrine markers (synaptophysin, chromogranin, CD56)
References:
1. Basturk O, Yang Z, Tang LH, Hruban RH, Adsay V, McCall CM, Krasinskas AM, Jang KT, Frankel WL, Balci S, Sigel C, Klimstra DS. The high-grade (WHO G3) pancreatic neuroendocrine tumor category is morphologically and biologically heterogenous and includes both well differentiated and poorly differentiated neoplasms. Am J Surg Pathol. 2015 May;39(5):683-90
2. McCall CM, Shi C, Cornish TC, Klimstra DS, Tang LH, Basturk O, Mun LJ, Ellison TA, Wolfgang CL, Choti MA, Schulick RD, Edil BH, Hruban RH. Grading of well-differentiated pancreatic neuroendocrine tumors is improved by the inclusion of both Ki67 proliferative index and mitotic rate. Am J Surg Pathol. 2013 Nov;37(11):1671-7
3. McCall CM, Shi C, Klein AP, Konukiewitz B, Edil BH, Ellison TA, Wolfgang CL, Schulick RD, Klöppel G, Hruban RH. Serotonin expression in pancreatic neuroendocrine tumors correlates with a trabecular histologic pattern and large duct involvement. Hum Pathol. 2012 Aug;43(8):1169-76.
4. Shi C, Siegelman SS, Kawamoto S, Wolfgang CL, Schulick RD, Maitra A, Hruban RH. Pancreatic duct stenosis secondary to small endocrine neoplasms: a manifestation of serotonin production? Radiology. 2010 Oct;257(1):107-14.
5. Tang LH, Basturk O, Sue JJ, Klimstra DS. A Practical Approach to the Classification of WHO Grade 3 (G3) Well-differentiated Neuroendocrine Tumor (WD-NET) and Poorly Differentiated Neuroendocrine Carcinoma (PD-NEC) of the Pancreas. Am J Surg Pathol. 2016 Sep;40(9):1192-202.
Histology: This pancreatic resection specimen displays areas of unremarkable pancreatic parenchyma, with separate regions displaying chronic pancreatitis, suggestive of ductal obstruction. Within the pancreas is a fibrotic tumor characterized by cells arranged in nests, trabeculae, single file, and single cell patterns. The cells have amphophilic granular cytoplasm and oval to rounded nuclei with finely dispersed “salt and pepper chromatin,” occasionally prominent nucleoli, and scattered degenerative-type atypia. Mitotic figures are inconspicuous. Perinerual invasion is readily identified, and metastatic foci are present within a peripancreatic lymph node. Immunotains show the lesional cells to be immunoreactive for synaptophysin and serotonin, and negative for BCL10, with a Ki67 proliferation index of <2%.
Discussion: This lesion exhibits the morphologic and immunophenotypic features of a well-differentiated, pancreatic neuroendocrine tumor (PanNET), which are further subdivided on the basis of tumor grade and (to an extent) tumor functionality. PanNETs are graded on the basis of the mitotic count per high power field (HPF) or the Ki67 proliferation index (measured as a percentage of cells with nuclear Ki67 labeling), and the higher of the two measures is used to determine the grade. Well-differentiated PanNETs display bland morphologic features (i.e., lack necrosis, lack features of small cell or large cell neuroendocrine carcinoma) and are classified as Grade 1 (mitoses <2/10 HPF or Ki67 <2%), Grade 2 (mitoses 2-20/10 HPF or Ki67 3-20%), or Grade 3 (mitoses >20/10 HPF or Ki67 >20%). Poorly differentiated pancreatic neuroendocrine carcinoma is also Grade 3 (mitoses >20/10 HPF or Ki67 >20%) but in addition displays histologic features of small cell carcinoma or large cell neuroendocrine carcinoma. The differential diagnosis of a solid cellular neoplasm in the pancreas includes acinar cell carcinoma, solid pseudopapillary neoplasms, metastases to the pancreas, and possibly ductal adenocarcinoma. In this particular tumor, there is abundant fibrosis, which can mimic the desmoplasia seen in ductal adenocarcinoma. However, the presence of the fibrosis as well as the trabecular growth pattern is a clue to the functional status of this tumor: it is a serotonin-secreting, well-differentiated grade 1 PanNET. Serotonin-secreting PanNETs characteristically show fibrosis, calcification, large duct involvement, and trabecular growth. Not all PanNETs are functional, but all should display some degree of labeling for neuroendocrine markers (synaptophysin, chromogranin, CD56)
References:
1. Basturk O, Yang Z, Tang LH, Hruban RH, Adsay V, McCall CM, Krasinskas AM, Jang KT, Frankel WL, Balci S, Sigel C, Klimstra DS. The high-grade (WHO G3) pancreatic neuroendocrine tumor category is morphologically and biologically heterogenous and includes both well differentiated and poorly differentiated neoplasms. Am J Surg Pathol. 2015 May;39(5):683-90
2. McCall CM, Shi C, Cornish TC, Klimstra DS, Tang LH, Basturk O, Mun LJ, Ellison TA, Wolfgang CL, Choti MA, Schulick RD, Edil BH, Hruban RH. Grading of well-differentiated pancreatic neuroendocrine tumors is improved by the inclusion of both Ki67 proliferative index and mitotic rate. Am J Surg Pathol. 2013 Nov;37(11):1671-7
3. McCall CM, Shi C, Klein AP, Konukiewitz B, Edil BH, Ellison TA, Wolfgang CL, Schulick RD, Klöppel G, Hruban RH. Serotonin expression in pancreatic neuroendocrine tumors correlates with a trabecular histologic pattern and large duct involvement. Hum Pathol. 2012 Aug;43(8):1169-76.
4. Shi C, Siegelman SS, Kawamoto S, Wolfgang CL, Schulick RD, Maitra A, Hruban RH. Pancreatic duct stenosis secondary to small endocrine neoplasms: a manifestation of serotonin production? Radiology. 2010 Oct;257(1):107-14.
5. Tang LH, Basturk O, Sue JJ, Klimstra DS. A Practical Approach to the Classification of WHO Grade 3 (G3) Well-differentiated Neuroendocrine Tumor (WD-NET) and Poorly Differentiated Neuroendocrine Carcinoma (PD-NEC) of the Pancreas. Am J Surg Pathol. 2016 Sep;40(9):1192-202.
Histology: This resection specimen shows distinct nodules with fibrous capsules and peripheral lymphocytes, with adjacent histologically unremarkable pancreatic parenchyma. The nodules are comprised of nests of cells with clear-to-fibrillary cytoplasm, atypical nuclei with focally prominent nucleoli, occasional mitotic figures, and numerous capillaries with “chicken wire” pattern. The lesional cells are diffusely positive for Pax8 and are negative for synaptophysin and chromogranin.
Discussion: This is a metastatic clear cell renal cell carcinoma (RCC) to the pancreas. The main differential diagnosis in this setting includes primary pancreatic neuroendocrine tumor (PanNET) with clear cell change. This differential diagnosis is particularly challenging and important because both clear cell RCC and PanNETs with clear cell change occur in patients with the von Hippel-Lindau (VHL) syndrome. VHL syndrome is due to autosomal dominant mutations in the VHL gene located on chromosome 3, and patients with VHL are susceptible not only to the former lesions, but also to hemangioblastomas, pheochromocytomas, retinal angiomas, endolymphatic sac tumors, and epididymal papillary cystadenomas, among other lesions. In addition, not only are patients with VHL susceptible to both clear cell RCCs and PanNETs with clear cell change, clear cell RCCs are among carcinoma types that metastasize to “unusual locations” such as the pancreas and thyroid. (However, clear cell RCCs do also metastasize to more “typical sites” of metastatic carcinomas, such as lung, adrenal, and brain). Clear cell RCC are also unusual in that they may metastasize after a long disease-free interval, such that the pathologist may not be aware of the clinical history. Immunostains can help resolve the differential between clear cell RCC and PanNET with clear cell change; however, there is a serious pitfall! Clear cell RCC are classically immunoreactive for Pax8, but PanNETs can also label with Pax8! Thus, immunostains for neuroendocrine differentiation, such as synaptophysin, chromogranin, and CD56 must be included in the immunopanel. In general, clear cell RCC are immunoreactive for Pax8, CAIX, CD10 and vimentin, and are negative for neuroendocrine markers. PanNETs, including those with clear cell change are immunoreactive for neuroendocrine markers and can be positive for Pax8.
References:
1. Hoang MP, Hruban RH, Albores-Saavedra J. Clear cell endocrine pancreatic tumor mimicking renal cell carcinoma: a distinctive neoplasm of von Hippel-Lindau disease. Am J Surg Pathol. 2001 May;25(5):602-9.
Histology: This resection specimen shows distinct nodules with fibrous capsules and peripheral lymphocytes, with adjacent histologically unremarkable pancreatic parenchyma. The nodules are comprised of nests of cells with clear-to-fibrillary cytoplasm, atypical nuclei with focally prominent nucleoli, occasional mitotic figures, and numerous capillaries with “chicken wire” pattern. The lesional cells are diffusely positive for Pax8 and are negative for synaptophysin and chromogranin.
Discussion: This is a metastatic clear cell renal cell carcinoma (RCC) to the pancreas. The main differential diagnosis in this setting includes primary pancreatic neuroendocrine tumor (PanNET) with clear cell change. This differential diagnosis is particularly challenging and important because both clear cell RCC and PanNETs with clear cell change occur in patients with the von Hippel-Lindau (VHL) syndrome. VHL syndrome is due to autosomal dominant mutations in the VHL gene located on chromosome 3, and patients with VHL are susceptible not only to the former lesions, but also to hemangioblastomas, pheochromocytomas, retinal angiomas, endolymphatic sac tumors, and epididymal papillary cystadenomas, among other lesions. In addition, not only are patients with VHL susceptible to both clear cell RCCs and PanNETs with clear cell change, clear cell RCCs are among carcinoma types that metastasize to “unusual locations” such as the pancreas and thyroid. (However, clear cell RCCs do also metastasize to more “typical sites” of metastatic carcinomas, such as lung, adrenal, and brain). Clear cell RCC are also unusual in that they may metastasize after a long disease-free interval, such that the pathologist may not be aware of the clinical history. Immunostains can help resolve the differential between clear cell RCC and PanNET with clear cell change; however, there is a serious pitfall! Clear cell RCC are classically immunoreactive for Pax8, but PanNETs can also label with Pax8! Thus, immunostains for neuroendocrine differentiation, such as synaptophysin, chromogranin, and CD56 must be included in the immunopanel. In general, clear cell RCC are immunoreactive for Pax8, CAIX, CD10 and vimentin, and are negative for neuroendocrine markers. PanNETs, including those with clear cell change are immunoreactive for neuroendocrine markers and can be positive for Pax8.
References:
1. Hoang MP, Hruban RH, Albores-Saavedra J. Clear cell endocrine pancreatic tumor mimicking renal cell carcinoma: a distinctive neoplasm of von Hippel-Lindau disease. Am J Surg Pathol. 2001 May;25(5):602-9.
Presented by Dr. Epstein and prepared by Austin McCuiston.
A 45 year old male was noted to have an asymptomatic 1 cm. testicular mass.
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A 45 year old male was noted to have an asymptomatic 1 cm. testicular mass.
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Answer: Large cell calcifying Sertoli cell tumor
Histology: The lesion is well-circumscribed. Areas of the tumor are fibrotic containing small nests of cells with abundant eosinophilic cytoplasm. Nuclei are uniform with central prominent nucleoli, lacking mitotic activity. The striking feature of this case is the dense areas of calcification with focal bone formation.
Discussion: This case shows the classic histology of large cell calcifying Sertoli cell tumor. There are no other tumors in the testis characterized by large areas of calcification. Although the cells resemble Leydig cells more than Sertoli cells, these tumors are considered the latter because occasionally there is tubular formation, and can occasionally show an intratubular component in adjacent seminiferous tubules. Most cases are sporadic but approximately 40% are associated with the Carney complex. Malignant tumors are uncommon and have the same criteria for malignancy as other Sertoli or Leydig cell tumors (>5 cm; >3 mitotic figures per 10 HPF; atypia; necrosis; lymphovascular invasion; very infiltrative growth). Immunohistochemically, they label like other sex cord stromal tumors with the most sensitive marker being SF1 and also inhibin.
Incorrect
Answer: Large cell calcifying Sertoli cell tumor
Histology: The lesion is well-circumscribed. Areas of the tumor are fibrotic containing small nests of cells with abundant eosinophilic cytoplasm. Nuclei are uniform with central prominent nucleoli, lacking mitotic activity. The striking feature of this case is the dense areas of calcification with focal bone formation.
Discussion: This case shows the classic histology of large cell calcifying Sertoli cell tumor. There are no other tumors in the testis characterized by large areas of calcification. Although the cells resemble Leydig cells more than Sertoli cells, these tumors are considered the latter because occasionally there is tubular formation, and can occasionally show an intratubular component in adjacent seminiferous tubules. Most cases are sporadic but approximately 40% are associated with the Carney complex. Malignant tumors are uncommon and have the same criteria for malignancy as other Sertoli or Leydig cell tumors (>5 cm; >3 mitotic figures per 10 HPF; atypia; necrosis; lymphovascular invasion; very infiltrative growth). Immunohistochemically, they label like other sex cord stromal tumors with the most sensitive marker being SF1 and also inhibin.
Presented by Dr. Epstein and prepared by Austin McCuiston.
A 36 year old male presented with hematuria and was noted to have a 4 mm lesion at cystoscopy.
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A 36 year old male presented with hematuria and was noted to have a 4 mm lesion at cystoscopy.
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Answer: Carcinoid tumor
Histology: Immediately below the urothelium are nests of urothelium are nests of bland urothelium with central lumen formation, some with apical cytoplasm. Beneath these nests are cribriform structures and tubules lined by uniform cells with eosinophilic cytoplasm and nuclei with delicate chromatin without atypia or mitoses.
Discussion: This case shows the typical appearance of a carcinoid of the bladder. They are usually small lesions that are benign and not infiltrative. As in this case, a prominent component is tubular formation. In contrast to true glands, the cells in the tubules are not lining the base of the cells with apical cytoplasm but rather are in the mid portion of the cells. Carcinoids of the bladder are frequently associated with proliferation of cystitis cystica et glandularis, present in this case also. Nephrogenic adenomas lack cribriform formation and their tubules are more atrophic than seen in this case. Rarely, carcinoid tumors of the bladder can be larger invasive tumors with the more classic appearance composed of ribbons and festoons.
Histology: Immediately below the urothelium are nests of urothelium are nests of bland urothelium with central lumen formation, some with apical cytoplasm. Beneath these nests are cribriform structures and tubules lined by uniform cells with eosinophilic cytoplasm and nuclei with delicate chromatin without atypia or mitoses.
Discussion: This case shows the typical appearance of a carcinoid of the bladder. They are usually small lesions that are benign and not infiltrative. As in this case, a prominent component is tubular formation. In contrast to true glands, the cells in the tubules are not lining the base of the cells with apical cytoplasm but rather are in the mid portion of the cells. Carcinoids of the bladder are frequently associated with proliferation of cystitis cystica et glandularis, present in this case also. Nephrogenic adenomas lack cribriform formation and their tubules are more atrophic than seen in this case. Rarely, carcinoid tumors of the bladder can be larger invasive tumors with the more classic appearance composed of ribbons and festoons.
Presented by Dr. Epstein and prepared by Austin McCuiston.
A man with several years of severe hypertension and end stage cardiomyopathy was scheduled for heart transplant when a 7 cm renal mass was noted.
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A man with several years of severe hypertension and end stage cardiomyopathy was scheduled for heart transplant when a 7 cm renal mass was noted.
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Answer: Juxtaglomerular cell tumor
Histology: The tumor consists of sheets of cells separated by a fine vascular network. The tumor cells have oval-round nuclei with uniform cytology, delicate chromatin, no mitotic figures admixed with a sprinkling of lymphocytes.
Discussion: Juxtaglomerular cell tumors are very rare. The tumor does not resemble any of the more common renal cell carcinoma variants. The morphology is typical for a juxtaglomerular cell tumor where is has some features of a glomus tumor but more ovoid nuclei and some features of a hemangiopericytoma in terms of the nuclear features and prominent vessels. Juxtaglomerular cell tumor can be confirmed by correlating with the clinical history. Patients almost always have uncontrollable and very high hypertension that is far beyond usual hypertension. Many patients are also females in the 20s and 30s, in whom such a history would be particularly uncommon. Characteristic renin crystals are seen ultrasctructurally but such studies are not required for the diagnosis. There is no commercially available immunohistochemical antibody for renin. Once the tumor is resected the hypertension almost always reverts to normal. With the exception of a single case report, these tumors are benign.
Incorrect
Answer: Juxtaglomerular cell tumor
Histology: The tumor consists of sheets of cells separated by a fine vascular network. The tumor cells have oval-round nuclei with uniform cytology, delicate chromatin, no mitotic figures admixed with a sprinkling of lymphocytes.
Discussion: Juxtaglomerular cell tumors are very rare. The tumor does not resemble any of the more common renal cell carcinoma variants. The morphology is typical for a juxtaglomerular cell tumor where is has some features of a glomus tumor but more ovoid nuclei and some features of a hemangiopericytoma in terms of the nuclear features and prominent vessels. Juxtaglomerular cell tumor can be confirmed by correlating with the clinical history. Patients almost always have uncontrollable and very high hypertension that is far beyond usual hypertension. Many patients are also females in the 20s and 30s, in whom such a history would be particularly uncommon. Characteristic renin crystals are seen ultrasctructurally but such studies are not required for the diagnosis. There is no commercially available immunohistochemical antibody for renin. Once the tumor is resected the hypertension almost always reverts to normal. With the exception of a single case report, these tumors are benign.
Presented by Dr. Matoso and prepared by Austin McCuiston.
43 yo female with kidney tumor.
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43 yo female with kidney tumor.
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Answer: Metanephric adenoma
Histology: This tumor is composed of primitive tubules with scattered poorly formed papillary structures and cord of cells with round nuclei, inconspicuous nucleoli and scant amount of cytoplasm. There is no mitotic activity, or tumor necrosis.
Discussion: This is an example of metanephric adenoma. These tumors show a female predominance and can happen at any age. They are usually non-encapsulated but this feature might not be evident in a needle biopsy as seen in this case. Psammomatous calcifications could be seen and the presence of necrosis or hemorrhage is rare and does not equate malignancy. The main differential diagnoses include papillary renal cell carcinoma and Wilms tumor. By immunohistochemistry, the tumor is positive for PAX8, WT1 and CD57 but negative for CK7 and CD56. In contrast, papillary renal cell carcinoma is frequently positive for CK7 and negative for WT1. Epithelial predominant Wilms tumors show a thick fibrous capsule and the tumor cells are more columnar with frequent mitoses. Wilms tumors are positive for CD56 and frequently negative for CD57. Metanephric adenomas frequently harbor BRAF mutation.
Histology: This tumor is composed of primitive tubules with scattered poorly formed papillary structures and cord of cells with round nuclei, inconspicuous nucleoli and scant amount of cytoplasm. There is no mitotic activity, or tumor necrosis.
Discussion: This is an example of metanephric adenoma. These tumors show a female predominance and can happen at any age. They are usually non-encapsulated but this feature might not be evident in a needle biopsy as seen in this case. Psammomatous calcifications could be seen and the presence of necrosis or hemorrhage is rare and does not equate malignancy. The main differential diagnoses include papillary renal cell carcinoma and Wilms tumor. By immunohistochemistry, the tumor is positive for PAX8, WT1 and CD57 but negative for CK7 and CD56. In contrast, papillary renal cell carcinoma is frequently positive for CK7 and negative for WT1. Epithelial predominant Wilms tumors show a thick fibrous capsule and the tumor cells are more columnar with frequent mitoses. Wilms tumors are positive for CD56 and frequently negative for CD57. Metanephric adenomas frequently harbor BRAF mutation.
Presented by Dr. Matoso and prepared by Austin McCuiston.
38 yo Man with testicular mass-frozen section diagnosis.
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38 yo Man with testicular mass-frozen section diagnosis.
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Answer: Adenomatoid tumor
Histology. This tumor shows a mass composed of tubules or glandular like structures surrounded by smooth muscle. The tubules are composed of bland cuboidal to flat cells with eosinophilic cytoplasm and vesicular nuclei.
Discussion: This is an example of an adenomatoid tumor. Adenomatoid tumor is the most common tumor of the testicular adnexa. They usually present as small growing mass intrascrotal tumor with a paratesticular location identified on image studies. It is not unusual to receive a frozen section in this situation as the urologists are trying to confirm the diagnosis to preserve the testis. The morphologic features that are characteristic include tumor composed of solid nests/cords and or tubules/poorly formed glands. The cells are either flat or cuboidal and have moderate amount of eosinophilic cytoplasm and vesicular nuclei. The cytoplasm may show prominent vacuolization giving it a signet ring cell appearance. Mitosis or necrosis are not seen. Adenomatoid tumors are usually surrounded by thick smooth muscle bundles that can be misinterpreted by stromal invasion. The main differential diagnoses are sex cord stromal tumors, especially Sertoli cell tumors due to the tubule formation, metastatic carcinoma and yolk sac tumor. Serologic markers are negative. Malignant mesothelioma should also be a consideration but it is usually much more cytologically atypical and shows desmoplastic stroma.
References:
Eur J Med Res. 2018 Jan 11;23(1):3.
Rev Urol. 2016;18(1):51-3.
Incorrect
Answer: Adenomatoid tumor
Histology. This tumor shows a mass composed of tubules or glandular like structures surrounded by smooth muscle. The tubules are composed of bland cuboidal to flat cells with eosinophilic cytoplasm and vesicular nuclei.
Discussion: This is an example of an adenomatoid tumor. Adenomatoid tumor is the most common tumor of the testicular adnexa. They usually present as small growing mass intrascrotal tumor with a paratesticular location identified on image studies. It is not unusual to receive a frozen section in this situation as the urologists are trying to confirm the diagnosis to preserve the testis. The morphologic features that are characteristic include tumor composed of solid nests/cords and or tubules/poorly formed glands. The cells are either flat or cuboidal and have moderate amount of eosinophilic cytoplasm and vesicular nuclei. The cytoplasm may show prominent vacuolization giving it a signet ring cell appearance. Mitosis or necrosis are not seen. Adenomatoid tumors are usually surrounded by thick smooth muscle bundles that can be misinterpreted by stromal invasion. The main differential diagnoses are sex cord stromal tumors, especially Sertoli cell tumors due to the tubule formation, metastatic carcinoma and yolk sac tumor. Serologic markers are negative. Malignant mesothelioma should also be a consideration but it is usually much more cytologically atypical and shows desmoplastic stroma.
References:
Eur J Med Res. 2018 Jan 11;23(1):3.
Rev Urol. 2016;18(1):51-3.
Presented by Dr. Matoso and prepared by Austin McCuiston.
87 yo F with urethral mass.
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87 yo F with urethral mass.
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Answer: Melanoma
Histology: This mass is composed of cytologically atpypical spindle cells haphazardly distributed with superficial erosion. There is brisk mitotic activity.
Discussion: Primary melanoma of the genitourinary tract is very rare but well documented. The most common site of origin is the urethra and represents less than 1% of all melanomas. It is more frequent in females than males with a female:male ratio of 3:1. Most commonly affects the distal urethra and urethral meatus. In men, more than half of the cases occur in the fosa navicularis, and the remaining are distributed in all different segments of the urethra, including in the prostatic urethra. The clinical presentation includes perineal mass and pain, dysuria, incontinence, hematuria or local bleeding. Occasionally, if the mass is occluding the lumen of the urethra, the presenting symptoms could be voiding dysfunction. One case had the diagnosis of lentigo of the meatus for several years. The clinical presentation might include inguinal lymphadenopathy, due to reactive lymphoid hyperplasia due to infection of the primary lesion of lymph node enlargement due to metastasis.
Microscopic Features: Histopathological examination most commonly reveals a polypoid tumor with overlying mucosal ulceration, partially covered with squamous mucosa. Tumor cells may be epithelioid or spindled and can be arranged in nests, or grow in a fascicular pattern and with large pleomorphic cells. The most common pattern of growth is diffuse followed by nested. Prominent rhabdoid features was observed in many cases. Pseudopapillary growth was also reported. Due to surface ulceration, there could be an inflammatory infiltrate. Melanin deposition is not always present. Mitoses and necrosis can be seen.
Differential Diagnosis: Epithelioid melanomas should be differentiated from carcinomas. In the urethra, the most frequent carcinomas include squamous and urothelial. Spindle cell melanomas could be misdiagnosed as sarcomatoid carcinoma. Neoplastic cells can show reactivity for or more of melanoma markers including Melan-A, HMB45, SOX10, MITF, MART-1 and S100 protein. Markers of urothelial or squamous differentiation including high molecular weight cytokeratin, p63 or GATA-3 are usually negative in melanomas. Metastatic melanoma to the urethra is rarely a clinically significant finding but it should be considered within the differential diagnosis. Of the patients included in the largest case series published, 2/15 (13%) had history of another melanoma in other location but were thought to have a new primary in the urethra.
Diagnosis: Almost invariably, the diagnosis of primary melanoma of the urinary tract involves the use of immunohistochemistry stains to prove the melanocytic origin of malignant cells. These markers include HMB45, Melan-A, MART1, MITF1, S100, SOX10. Due to its rarity, there are no well-established guidelines of histopathologic parameters that are expected to be included in the pathology report. Some basic information that should be mentioned in the report include size, depth of invasion (pathologic stage), vascular or perineural invasion, mitotic activity and distance to margins.
Prognosis: Overall, primary melanoma of the urethra has a poor prognosis with high recurrence rates and systemic metastases. The management options include radical urethrectomy followed by radiotherapy and/or systemic chemotherapy. A study that investigated mutations in genitourinary melanomas in women identified one of five urethral melanomas with an NRAS mutation and none with a KIT or BRAF mutation.
References:
1. McComiskey M, Iavazzo C, Datta M, et al. Balloon Cell Urethral Melanoma: Differential Diagnosis and Management. Case Rep Obstet Gynecol. 2015;2015:919584.
2. DeSimone RA, Hoda RS. Primary malignant melanoma of the urethra detected by urine cytology in a male patient. Diagn Cytopathol. 2015;43(8):680-682.
3. Dasgupta T, Grabstald H. Melanoma of the Genitourinary Tract. J Urol. 1965;93:607-614.
4. Bhutani N, Kajal P, Pawar D. Primary malignant melanoma of the female urethra: Report of a rare neoplasm of the urinary tract. Int J Surg Case Rep. 2017;41:319-322.
5. Oliva E, Quinn TR, Amin MB, et al. Primary malignant melanoma of the urethra: a clinicopathologic analysis of 15 cases. Am J Surg Pathol. 2000;24(6):785-796.
6. Heslinga JM, Lycklama a Nijeholt GA, Ruiter DJ. Primary melanoma in the female distal urethra. Eur Urol. 1986;12(6):446-447.
Incorrect
Answer: Melanoma
Histology: This mass is composed of cytologically atpypical spindle cells haphazardly distributed with superficial erosion. There is brisk mitotic activity.
Discussion: Primary melanoma of the genitourinary tract is very rare but well documented. The most common site of origin is the urethra and represents less than 1% of all melanomas. It is more frequent in females than males with a female:male ratio of 3:1. Most commonly affects the distal urethra and urethral meatus. In men, more than half of the cases occur in the fosa navicularis, and the remaining are distributed in all different segments of the urethra, including in the prostatic urethra. The clinical presentation includes perineal mass and pain, dysuria, incontinence, hematuria or local bleeding. Occasionally, if the mass is occluding the lumen of the urethra, the presenting symptoms could be voiding dysfunction. One case had the diagnosis of lentigo of the meatus for several years. The clinical presentation might include inguinal lymphadenopathy, due to reactive lymphoid hyperplasia due to infection of the primary lesion of lymph node enlargement due to metastasis.
Microscopic Features: Histopathological examination most commonly reveals a polypoid tumor with overlying mucosal ulceration, partially covered with squamous mucosa. Tumor cells may be epithelioid or spindled and can be arranged in nests, or grow in a fascicular pattern and with large pleomorphic cells. The most common pattern of growth is diffuse followed by nested. Prominent rhabdoid features was observed in many cases. Pseudopapillary growth was also reported. Due to surface ulceration, there could be an inflammatory infiltrate. Melanin deposition is not always present. Mitoses and necrosis can be seen.
Differential Diagnosis: Epithelioid melanomas should be differentiated from carcinomas. In the urethra, the most frequent carcinomas include squamous and urothelial. Spindle cell melanomas could be misdiagnosed as sarcomatoid carcinoma. Neoplastic cells can show reactivity for or more of melanoma markers including Melan-A, HMB45, SOX10, MITF, MART-1 and S100 protein. Markers of urothelial or squamous differentiation including high molecular weight cytokeratin, p63 or GATA-3 are usually negative in melanomas. Metastatic melanoma to the urethra is rarely a clinically significant finding but it should be considered within the differential diagnosis. Of the patients included in the largest case series published, 2/15 (13%) had history of another melanoma in other location but were thought to have a new primary in the urethra.
Diagnosis: Almost invariably, the diagnosis of primary melanoma of the urinary tract involves the use of immunohistochemistry stains to prove the melanocytic origin of malignant cells. These markers include HMB45, Melan-A, MART1, MITF1, S100, SOX10. Due to its rarity, there are no well-established guidelines of histopathologic parameters that are expected to be included in the pathology report. Some basic information that should be mentioned in the report include size, depth of invasion (pathologic stage), vascular or perineural invasion, mitotic activity and distance to margins.
Prognosis: Overall, primary melanoma of the urethra has a poor prognosis with high recurrence rates and systemic metastases. The management options include radical urethrectomy followed by radiotherapy and/or systemic chemotherapy. A study that investigated mutations in genitourinary melanomas in women identified one of five urethral melanomas with an NRAS mutation and none with a KIT or BRAF mutation.
References:
1. McComiskey M, Iavazzo C, Datta M, et al. Balloon Cell Urethral Melanoma: Differential Diagnosis and Management. Case Rep Obstet Gynecol. 2015;2015:919584.
2. DeSimone RA, Hoda RS. Primary malignant melanoma of the urethra detected by urine cytology in a male patient. Diagn Cytopathol. 2015;43(8):680-682.
3. Dasgupta T, Grabstald H. Melanoma of the Genitourinary Tract. J Urol. 1965;93:607-614.
4. Bhutani N, Kajal P, Pawar D. Primary malignant melanoma of the female urethra: Report of a rare neoplasm of the urinary tract. Int J Surg Case Rep. 2017;41:319-322.
5. Oliva E, Quinn TR, Amin MB, et al. Primary malignant melanoma of the urethra: a clinicopathologic analysis of 15 cases. Am J Surg Pathol. 2000;24(6):785-796.
6. Heslinga JM, Lycklama a Nijeholt GA, Ruiter DJ. Primary melanoma in the female distal urethra. Eur Urol. 1986;12(6):446-447.
Histologic Description: Large portions of this lesion are entirely consistent with a grade 1 meningioma, in that the lesional cells are bland and form well-developed whorls. Some nuclei, which are bland, demonstrate characteristic intranuclear inclusions. However, associated with the meningioma is a high grade adenocarcinoma with extensive necrosis. This represents the metastases of the patient’s concurrent duodenal adenocarcinoma.
Differential Diagnosis: Sarcomatoid carcinoma would demonstrate greater atypia in the spindle cell component, and would not demonstrate the characteristic morphology of meningioma. Malignant meningiomas are characterized by increased mitotic activity and sheet like growth, but gland formation would not be typical. The diagnosis of metastatic adenocarcinoma alone would ignore the meningioma component of the current lesion.
Meningioma is a frequent recipient of metastasis, particularly breast cancer.
Histologic Description: Large portions of this lesion are entirely consistent with a grade 1 meningioma, in that the lesional cells are bland and form well-developed whorls. Some nuclei, which are bland, demonstrate characteristic intranuclear inclusions. However, associated with the meningioma is a high grade adenocarcinoma with extensive necrosis. This represents the metastases of the patient’s concurrent duodenal adenocarcinoma.
Differential Diagnosis: Sarcomatoid carcinoma would demonstrate greater atypia in the spindle cell component, and would not demonstrate the characteristic morphology of meningioma. Malignant meningiomas are characterized by increased mitotic activity and sheet like growth, but gland formation would not be typical. The diagnosis of metastatic adenocarcinoma alone would ignore the meningioma component of the current lesion.
Meningioma is a frequent recipient of metastasis, particularly breast cancer.
Presented by Dr. Argani and prepared by Austin McCuiston
This is a 57 year old male with pleural mass
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This is a 57 year old male with pleural mass.
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Answer: Schwannoma
Histologic Description: This is a well delineated spindle cell neoplasm. The lesion demonstrates variable cellularity. The neoplastic cells have angulated, “club-shaped” nuclei, and focally demonstrate palisading consistent with Verocay body formation. Other areas of the lesion are hypocellular and associated with ectatic blood vessels. The lesion was diffusely immunoreactive for S100 protein, supporting the diagnosis of schwannoma.
Differential Diagnosis: Solitary fibrous tumor would demonstrate more hemangiopericytomatous vasculature, greater collagen production, and would be diffusely positive for CD34 but not S100. Sarcomatoid mesothelioma would not be well delineated, would demonstrate greater cytologic atypia, and label diffusely for cytokeratin. Thymoma frequently metastasizes to the pleura; however, the spindle cells would be associated with immature lymphocytes and label diffusely for cytokeratin and p63.
Incorrect
Answer: Schwannoma
Histologic Description: This is a well delineated spindle cell neoplasm. The lesion demonstrates variable cellularity. The neoplastic cells have angulated, “club-shaped” nuclei, and focally demonstrate palisading consistent with Verocay body formation. Other areas of the lesion are hypocellular and associated with ectatic blood vessels. The lesion was diffusely immunoreactive for S100 protein, supporting the diagnosis of schwannoma.
Differential Diagnosis: Solitary fibrous tumor would demonstrate more hemangiopericytomatous vasculature, greater collagen production, and would be diffusely positive for CD34 but not S100. Sarcomatoid mesothelioma would not be well delineated, would demonstrate greater cytologic atypia, and label diffusely for cytokeratin. Thymoma frequently metastasizes to the pleura; however, the spindle cells would be associated with immature lymphocytes and label diffusely for cytokeratin and p63.
Presented by Dr. Argani and prepared by Austin McCuiston.
This is a 4 week old male who undergoes an omental biopsy
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This is a 4 week old male who undergoes an omental biopsy.
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Answer: Fat necrosis
Histologic Description: This tissue consists of atrophic fat associated with mesothelial cells, consistent with omental tissue. The fat demonstrates uniform myxoid change with prominent capillary vasculature, simulating the morphology of myxoid liposarcoma. The changes are microscopic throughout the specimen, affecting virtually every fat lobule, and no mass lesion was identified. These features support the diagnosis of fat necrosis. This child was extremely ill which fits the picture
Differential Diagnosis: Well differentiated liposarcoma occurs in adults and would feature atypical, hyperchromatic lipoblasts in fibrous septa. Lipoblastoma resembles the current lesion; however, there is typically a mass lesion present, and the more immature appearing fat is at the edge of the lobules of the lesion. Myxoid liposarcoma similarly makes a mass lesion and demonstrates greater cytologic atypia.
Incorrect
Answer: Fat necrosis
Histologic Description: This tissue consists of atrophic fat associated with mesothelial cells, consistent with omental tissue. The fat demonstrates uniform myxoid change with prominent capillary vasculature, simulating the morphology of myxoid liposarcoma. The changes are microscopic throughout the specimen, affecting virtually every fat lobule, and no mass lesion was identified. These features support the diagnosis of fat necrosis. This child was extremely ill which fits the picture
Differential Diagnosis: Well differentiated liposarcoma occurs in adults and would feature atypical, hyperchromatic lipoblasts in fibrous septa. Lipoblastoma resembles the current lesion; however, there is typically a mass lesion present, and the more immature appearing fat is at the edge of the lobules of the lesion. Myxoid liposarcoma similarly makes a mass lesion and demonstrates greater cytologic atypia.
Presented by Dr. Jonathan Epstein and prepared by Austin McCuiston.
A 67 year old man underwent a nephrectomy for a renal mass.
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A 67 year old man underwent a nephrectomy for a renal mass.
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Answer: Carcinoid tumor
Histology: The tumor has a relatively uniform growth pattern consisting of cords and trabeculae. The tumor appears very vascular with numerous fine capillaries interspersed throughout. Cytologically, the cells are not very pleomorphic and lack prominent nucleoli with a fine speckled chromatin. In areas of the tumor there are dense fibrous bands. Mitotic figures are difficult to identify.
Discussion. The morphology of this tumor is classic for a carcinoid tumor. It is only difficult in that carcinoid tumors of the kidney are rare, and consequently pathologists do not think of it in the differential diagnosis of a renal tumor. Carcinoid tumors of the kidney are aggressive tumors with approximately 50% of them developing metastatic disease regardless of their mitotic count and even if necrosis is absent. Consequently, terminology used for these tumors in the kidney do not parallel that used in other organs where neuroendocrine tumors are divided into well-differentiated NE tumors and high grade NE carcinomas. Given that in the kidney, mitotic figures and necrosis do not correlate with prognosis, they are merely referred to as “carcinoid tumor” of the kidney where their prognosis is unpredictable but fairly aggressive.
Incorrect
Answer: Carcinoid tumor
Histology: The tumor has a relatively uniform growth pattern consisting of cords and trabeculae. The tumor appears very vascular with numerous fine capillaries interspersed throughout. Cytologically, the cells are not very pleomorphic and lack prominent nucleoli with a fine speckled chromatin. In areas of the tumor there are dense fibrous bands. Mitotic figures are difficult to identify.
Discussion. The morphology of this tumor is classic for a carcinoid tumor. It is only difficult in that carcinoid tumors of the kidney are rare, and consequently pathologists do not think of it in the differential diagnosis of a renal tumor. Carcinoid tumors of the kidney are aggressive tumors with approximately 50% of them developing metastatic disease regardless of their mitotic count and even if necrosis is absent. Consequently, terminology used for these tumors in the kidney do not parallel that used in other organs where neuroendocrine tumors are divided into well-differentiated NE tumors and high grade NE carcinomas. Given that in the kidney, mitotic figures and necrosis do not correlate with prognosis, they are merely referred to as “carcinoid tumor” of the kidney where their prognosis is unpredictable but fairly aggressive.
Presented by Dr. Jonathan Epstein and prepared by Austin McCuiston.
A 65 year old female underwent a partial excision for a renal mass.
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A 65 year old female underwent a partial excision for a renal mass.
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Answer: Oncocytoma
Histology: The architecture and cytology in this case is typical of oncocytoma. The tumor is arranged in discrete nests as opposed to the more sheet-like growth in chromophobe RCC. Cytologically, there are large, round-to-polygonal cells with abundant eosinophilic cytoplasm. Well-preserved nuclei that with fine chromatin are uniformly round with variably sized nucleoli. At the perimeter of the tumor, the lesion extends out into the peri-renal adipose tissue without inducing a stromal reaction. There is a central scar where the cells lose their oncocytic appearance and have more clear scant cytoplasm.
Discussion: Extra-renal extension into peri-renal adipose tissue may be seen in 20% of oncocytomas. Invasion of renal vessels including the main renal vein can also be rarely seen. Both of these features do not affect the 100% benign nature of oncocytomas. In contrast to chromophobe RCC, oncocytomas lack raisenoid nuclei with irregular, wrinkled, notched nuclear membranes and perinuclear halos, yet not every cell will have irregular nuclei. CK7 tends to be diffuse in chromophobe but can be negative or patchy. CK7 is patchy in oncocytomas and never diffuse. A pitfall is the tubules in the central scar of oncocytomas are typically diffuse CK7 positive. Hybrid tumor is used when there are discrete classic areas of chromophobe RCC and oncocytoma in the same tumor.
Incorrect
Answer: Oncocytoma
Histology: The architecture and cytology in this case is typical of oncocytoma. The tumor is arranged in discrete nests as opposed to the more sheet-like growth in chromophobe RCC. Cytologically, there are large, round-to-polygonal cells with abundant eosinophilic cytoplasm. Well-preserved nuclei that with fine chromatin are uniformly round with variably sized nucleoli. At the perimeter of the tumor, the lesion extends out into the peri-renal adipose tissue without inducing a stromal reaction. There is a central scar where the cells lose their oncocytic appearance and have more clear scant cytoplasm.
Discussion: Extra-renal extension into peri-renal adipose tissue may be seen in 20% of oncocytomas. Invasion of renal vessels including the main renal vein can also be rarely seen. Both of these features do not affect the 100% benign nature of oncocytomas. In contrast to chromophobe RCC, oncocytomas lack raisenoid nuclei with irregular, wrinkled, notched nuclear membranes and perinuclear halos, yet not every cell will have irregular nuclei. CK7 tends to be diffuse in chromophobe but can be negative or patchy. CK7 is patchy in oncocytomas and never diffuse. A pitfall is the tubules in the central scar of oncocytomas are typically diffuse CK7 positive. Hybrid tumor is used when there are discrete classic areas of chromophobe RCC and oncocytoma in the same tumor.
Presented by Dr. Jonathan Epstein and prepared by Austin McCuiston.
A 45 year old man was noted to have a diffusely enlarged testicle that was abnormal on ultrasound.
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A 45 year old man was noted to have a diffusely enlarged testicle that was abnormal on ultrasound. An orchiectomy was performed. The lesion was confined to the testis with a normal epididymis. Choose the correct diagnosis.
Correct
Answer: Idiopathic granulomatous orchitis
Histology: At low there are non necrotizing granulomas filling seminiferous tubules. Granulomas are composed of epithelioid histiocytes, giant cells admixed with lymphocytes and plasma cells. Chronic inflammatory cells including eosinophils infiltrate interstitium.
Discussion: With infectious granulomas, they are frequently initiated in the epididymis and spread to testis in later stages. In infectious processes, necrotizing and/or non-necrotizing granulomatous inflammation composed of epithelioid histiocytes and multinucleated giant cells mainly involving the testicular interstitium and not the seminiferous tubules. The granulomas are variable in size and distribution, in contrast to the uniform size and distribution of granulomas in idiopathic granulomatous orchitis (IGO) reflecting the filling up of pre-existing seminiferous tubules. The same interstitial distribution is also seen in sarcoidosis, yet non-necrotizing granulomas are seen. In some cases of seminoma there are intratubular seminoma cells which, just as seen in invasive seminoma, can elicit a granulomatous reaction. In almost all such case, there will be invasive seminoma seen which is lacking in IGO. In cases with invasive seminoma and intratubular epithelioid histiocytes, there are typically fewer giant cells than in IGO. The intratubular seminoma cells are enlarged, atypical germ cells with clear cytoplasm, irregular nuclear contours, coarse chromatin, and enlarged single or multiple nucleoli. Some seminoma cells have mummified pyknotic enlarged hyperchromatic nuclei. We have recently shown that IGO is not an IGG4 related disease and its pathogenesis remains unknown. IGO is a benign inflammatory condition but may lead to loss of spermatogenesis in the involved testis even if orchiectomy is avoided.
Incorrect
Answer: Idiopathic granulomatous orchitis
Histology: At low there are non necrotizing granulomas filling seminiferous tubules. Granulomas are composed of epithelioid histiocytes, giant cells admixed with lymphocytes and plasma cells. Chronic inflammatory cells including eosinophils infiltrate interstitium.
Discussion: With infectious granulomas, they are frequently initiated in the epididymis and spread to testis in later stages. In infectious processes, necrotizing and/or non-necrotizing granulomatous inflammation composed of epithelioid histiocytes and multinucleated giant cells mainly involving the testicular interstitium and not the seminiferous tubules. The granulomas are variable in size and distribution, in contrast to the uniform size and distribution of granulomas in idiopathic granulomatous orchitis (IGO) reflecting the filling up of pre-existing seminiferous tubules. The same interstitial distribution is also seen in sarcoidosis, yet non-necrotizing granulomas are seen. In some cases of seminoma there are intratubular seminoma cells which, just as seen in invasive seminoma, can elicit a granulomatous reaction. In almost all such case, there will be invasive seminoma seen which is lacking in IGO. In cases with invasive seminoma and intratubular epithelioid histiocytes, there are typically fewer giant cells than in IGO. The intratubular seminoma cells are enlarged, atypical germ cells with clear cytoplasm, irregular nuclear contours, coarse chromatin, and enlarged single or multiple nucleoli. Some seminoma cells have mummified pyknotic enlarged hyperchromatic nuclei. We have recently shown that IGO is not an IGG4 related disease and its pathogenesis remains unknown. IGO is a benign inflammatory condition but may lead to loss of spermatogenesis in the involved testis even if orchiectomy is avoided.
Presented by Dr. Argani and prepared by Austin McCuiston.
This is a 2 year old female with a brain mass that is immunoreactive for CD117 and e-cadherin.
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This is a 2 year old female with a brain mass that is immunoreactive for CD117 and e-cadherin.
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Answer: Erythroleukemia
Histologic Description: This is a typical primitive small round blue cell neoplasm that involves the brain. The cells focally have eccentric cytoplasm that is somewhat eosinophilic, raising the possibility of rhabdomyosarcoma. The cells are highly discohesive, which suggests hematopoietic origin. In addition to CD117 and e-cadherin, these neoplastic cells labeled for CD43 and spectrin, supporting the diagnosis of erythroleukemia.
Differential Diagnosis: Mast cell neoplasm could be suggested by CD117 immunoreactivity, but it is excluded by the labeling for the erythroid marker spectrin. E-cadherin raises the possibility of carcinoma; however, it is also a good marker of primitive erythroid neoplasms and not specific for epithelial lineage. Ewing sarcoma can be suggested in particular because erythroleukemia can label for CD99. However, the immunoreactivity for CD43 and spectrin excludes this diagnosis. Erythroleukemia has a tendency to present as extramedullary disease which mimics the other small round blue cell tumors of childhood, particularly when it labels for CD99.
Incorrect
Answer: Erythroleukemia
Histologic Description: This is a typical primitive small round blue cell neoplasm that involves the brain. The cells focally have eccentric cytoplasm that is somewhat eosinophilic, raising the possibility of rhabdomyosarcoma. The cells are highly discohesive, which suggests hematopoietic origin. In addition to CD117 and e-cadherin, these neoplastic cells labeled for CD43 and spectrin, supporting the diagnosis of erythroleukemia.
Differential Diagnosis: Mast cell neoplasm could be suggested by CD117 immunoreactivity, but it is excluded by the labeling for the erythroid marker spectrin. E-cadherin raises the possibility of carcinoma; however, it is also a good marker of primitive erythroid neoplasms and not specific for epithelial lineage. Ewing sarcoma can be suggested in particular because erythroleukemia can label for CD99. However, the immunoreactivity for CD43 and spectrin excludes this diagnosis. Erythroleukemia has a tendency to present as extramedullary disease which mimics the other small round blue cell tumors of childhood, particularly when it labels for CD99.
Presented by Dr. Argani and prepared by Austin McCuiston.
This is a 43 year old male with multiple pleural nodules.
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This is a 43 year old male with multiple pleural nodules.
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Answer: Metastatic thymoma
Histologic Description: The pleural nodules are characterized by dominant infiltrate of immature lymphocytes, which raises the differential diagnosis of small cell malignancies such as small cell carcinoma or, more significantly, lymphoblastic lymphoma. However, interdigitating between the dominant lymphoid components are epithelial cells with squamoid features and prominent processes, suggesting the possibility of thymoma. The epithelial cells are cytologically bland and lack malignant features. The epithelioid cells label for cytokeratin in a dendritic pattern, where the immature lymphocytes label for CD99 and TDT. The features are diagnostic of thymoma.
Differential Diagnosis: Small cell carcinoma would be expected to show cytokeratin labeling of the primitive small cells. Lymphoblastic lymphoma may have a phenotype similar to that of the immature lymphocytes of thymoma; however, lymphoblastic lymphoma would lack the dominant epithelial component of thymoma, and it occurs in a younger age group. Thymic carcinoma demonstrates cytologic features of malignancy in the epithelial component.
The current patient had a mediastinal mass, so this represents drop metastasis of thymoma.
Incorrect
Answer: Metastatic thymoma
Histologic Description: The pleural nodules are characterized by dominant infiltrate of immature lymphocytes, which raises the differential diagnosis of small cell malignancies such as small cell carcinoma or, more significantly, lymphoblastic lymphoma. However, interdigitating between the dominant lymphoid components are epithelial cells with squamoid features and prominent processes, suggesting the possibility of thymoma. The epithelial cells are cytologically bland and lack malignant features. The epithelioid cells label for cytokeratin in a dendritic pattern, where the immature lymphocytes label for CD99 and TDT. The features are diagnostic of thymoma.
Differential Diagnosis: Small cell carcinoma would be expected to show cytokeratin labeling of the primitive small cells. Lymphoblastic lymphoma may have a phenotype similar to that of the immature lymphocytes of thymoma; however, lymphoblastic lymphoma would lack the dominant epithelial component of thymoma, and it occurs in a younger age group. Thymic carcinoma demonstrates cytologic features of malignancy in the epithelial component.
The current patient had a mediastinal mass, so this represents drop metastasis of thymoma.
Presented by Dr. Argani and prepared by Austin McCuiston.
This is a 63 year old male with multiple liver lesions.
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This is a 63 year old male with multiple liver lesions.
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Answer: Epithelioid hemangioendothelioma
Histologic Description: This is a multifocal lesion which has prominent myxochondroid stroma and cords of epithelioid cells with cytoplasmic vacuolization, which raises the differential diagnosis of intrahepatic cholangiocarcinoma and metastatic adenocarcinoma. The vacuoles are focally septate, and there is no overt formation of blood vessels or of true glands. The neoplastic cells label for vascular markers CD34, CD31 and ERG, and demonstrate no immunoreactivity for cytokeratins. The findings are diagnostic of epithelioid hemangioendothelioma.
Differential Diagnosis: Both intrahepatic cholangiocarcinoma and metastatic adenocarcinoma would be expected to show strong immunoreactivity for cytokeratin. Hepatocellular carcinoma is infrequently sclerosing and typically features cells with more prominent granular eosinophilic cytoplasm.
Epithelioid hemangioendothelioma frequently shows focal cytokeratin immunoreactivity, which heightens the mimicry of adenocarcinoma. This lesion demonstrates a characteristic t(1;3) (p36;q25) chromosome translocation resulting in a WWTR1-CAMTA1 gene fusion.
Incorrect
Answer: Epithelioid hemangioendothelioma
Histologic Description: This is a multifocal lesion which has prominent myxochondroid stroma and cords of epithelioid cells with cytoplasmic vacuolization, which raises the differential diagnosis of intrahepatic cholangiocarcinoma and metastatic adenocarcinoma. The vacuoles are focally septate, and there is no overt formation of blood vessels or of true glands. The neoplastic cells label for vascular markers CD34, CD31 and ERG, and demonstrate no immunoreactivity for cytokeratins. The findings are diagnostic of epithelioid hemangioendothelioma.
Differential Diagnosis: Both intrahepatic cholangiocarcinoma and metastatic adenocarcinoma would be expected to show strong immunoreactivity for cytokeratin. Hepatocellular carcinoma is infrequently sclerosing and typically features cells with more prominent granular eosinophilic cytoplasm.
Epithelioid hemangioendothelioma frequently shows focal cytokeratin immunoreactivity, which heightens the mimicry of adenocarcinoma. This lesion demonstrates a characteristic t(1;3) (p36;q25) chromosome translocation resulting in a WWTR1-CAMTA1 gene fusion.
Presented by Dr. Jonathan Epstein and prepared by Austin McCuiston.
A 65 year old male was incidentally found to have a 5 cm renal mass on imaging.
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A 65 year old male was incidentally found to have a 5 cm renal mass on imaging.
Correct
Answer: Papillary renal cell carcinoma
Histological Description: The tumor is composed of large, round to polygonal cells with abundant eosinophilic cytoplasm. Architecturally, there are numerous papillary fronds lined by multilayered columnar granular eosinophilic/oncocytic cells. The nuclei are relatively round with prominent nucleoli. Numerous foamy histiocytes occupy the papillary fibrovascular cores. Hemosiderin deposition is present in some of the histiocytes as well in some of the epithelium.
Discussion: Certain features are not allowed in oncocytomas. They are: 1) papillary fronds; 2) necrosis; 3) clear cells (except for the degenerative cells in central scars); and 4) non-degenerative nuclear atypia consisting of irregular, notched, crinkly, folded nuclei. The one clarification about papillary features is that papillary projections may be seen into dilated cysts of oncocytomas composed of dilated tubules. Immunohistochemically, papillary renal cell carcinomas are typically diffusely positive for CK7. Oncocytomas can show patchy focal staining but never diffuse staining. Foamy histiocytes are a good clue in a renal tumor that it is papillary renal cell carcinoma and is not seen in oncocytomas. However, foamy histiocytes may be seen in other renal tumors so is not entirely specific. Another feature typical of papillary renal cell carcinoma is the presence of hemosiderin in tumor cells, also not seen in oncocytomas.
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Answer: Papillary renal cell carcinoma
Histological Description: The tumor is composed of large, round to polygonal cells with abundant eosinophilic cytoplasm. Architecturally, there are numerous papillary fronds lined by multilayered columnar granular eosinophilic/oncocytic cells. The nuclei are relatively round with prominent nucleoli. Numerous foamy histiocytes occupy the papillary fibrovascular cores. Hemosiderin deposition is present in some of the histiocytes as well in some of the epithelium.
Discussion: Certain features are not allowed in oncocytomas. They are: 1) papillary fronds; 2) necrosis; 3) clear cells (except for the degenerative cells in central scars); and 4) non-degenerative nuclear atypia consisting of irregular, notched, crinkly, folded nuclei. The one clarification about papillary features is that papillary projections may be seen into dilated cysts of oncocytomas composed of dilated tubules. Immunohistochemically, papillary renal cell carcinomas are typically diffusely positive for CK7. Oncocytomas can show patchy focal staining but never diffuse staining. Foamy histiocytes are a good clue in a renal tumor that it is papillary renal cell carcinoma and is not seen in oncocytomas. However, foamy histiocytes may be seen in other renal tumors so is not entirely specific. Another feature typical of papillary renal cell carcinoma is the presence of hemosiderin in tumor cells, also not seen in oncocytomas.
Presented by Dr. Jonathan Epstein and prepared by Austin McCuiston.
A 45 year old female was incidentally found to have a 3 cm renal mass on imaging.
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A 45 year old female was incidentally found to have a 3 cm renal mass on imaging.
Correct
Answer: Oncocytoma
Histology: The tumor is composed of cells with abundant eosinophilic cytoplasm. Focally, cells are arranged in nests in a fibromyxoid stroma. In this area the well-preserved nuclei that with fine chromatin are uniformly round with variably sized nucleoli. However, the majority of cells have prominent atypia, yet the atypia appears degenerative nuclear atypia with multinucleation, pseudoinclusions, and indistinct smudgy chromatin. Mitotic figures and necrosis are absent.
Discussion: One of the most characteristic features of oncocytoma is that cells are arranged in nests, acini, tubules or microcysts in a fibromyxoid stroma. When the tumor has this pattern at low power, 95% of the time the diagnosis is oncocytoma. In order for me to diagnose oncocytoma, well-preserved nuclei should be uniformly round with variably sized nucleoli. Pyknotic dark nuclei with crenated irregular edges should be discounted. Scattered cells with degenerative nuclear atypia with multinucleation and pseudoinclusions can be seen in a typical oncocytoma, where the atypical cells are often clustered. This case is unusual in that the atypical cells are more diffusely spread throughout the tumor. However, if this tumor was a high grade renal cell carcinoma with this degree of atypia, one would expect to see numerous mitotic figures and necrosis which are absent. Similarly, a ki67 in this case was very low, in contrast to what would be seen in a high grade renal cell carcinoma. In addition to degenerative nuclear atypia, oncocytomas can extend into the perirenal fat and can show vascular invasion, including the main renal vein, and still be benign. Chromophobe renal cell carcinomas can uncommonly be high grade with increased cellularity, necrosis, and increased mitotic figures, but retains typical cytological features of plant-like cell borders, crinkly nuclei surrounded by koilocytosis. Immunohistochemically, CK7 tends to be diffuse in chromophobe renal cell carcinoma and is negative to patchy in oncocytoma. However, some chromophobe carcinomas are patchy or negative for CK7, such that the only definitive result is that diffuse CK7 positivity rules out oncocytoma. Hales colloidal iron is often not specific and I do not rely on it, but in the classic case shows diffuse cytoplasmic staining.
Incorrect
Answer: Oncocytoma
Histology: The tumor is composed of cells with abundant eosinophilic cytoplasm. Focally, cells are arranged in nests in a fibromyxoid stroma. In this area the well-preserved nuclei that with fine chromatin are uniformly round with variably sized nucleoli. However, the majority of cells have prominent atypia, yet the atypia appears degenerative nuclear atypia with multinucleation, pseudoinclusions, and indistinct smudgy chromatin. Mitotic figures and necrosis are absent.
Discussion: One of the most characteristic features of oncocytoma is that cells are arranged in nests, acini, tubules or microcysts in a fibromyxoid stroma. When the tumor has this pattern at low power, 95% of the time the diagnosis is oncocytoma. In order for me to diagnose oncocytoma, well-preserved nuclei should be uniformly round with variably sized nucleoli. Pyknotic dark nuclei with crenated irregular edges should be discounted. Scattered cells with degenerative nuclear atypia with multinucleation and pseudoinclusions can be seen in a typical oncocytoma, where the atypical cells are often clustered. This case is unusual in that the atypical cells are more diffusely spread throughout the tumor. However, if this tumor was a high grade renal cell carcinoma with this degree of atypia, one would expect to see numerous mitotic figures and necrosis which are absent. Similarly, a ki67 in this case was very low, in contrast to what would be seen in a high grade renal cell carcinoma. In addition to degenerative nuclear atypia, oncocytomas can extend into the perirenal fat and can show vascular invasion, including the main renal vein, and still be benign. Chromophobe renal cell carcinomas can uncommonly be high grade with increased cellularity, necrosis, and increased mitotic figures, but retains typical cytological features of plant-like cell borders, crinkly nuclei surrounded by koilocytosis. Immunohistochemically, CK7 tends to be diffuse in chromophobe renal cell carcinoma and is negative to patchy in oncocytoma. However, some chromophobe carcinomas are patchy or negative for CK7, such that the only definitive result is that diffuse CK7 positivity rules out oncocytoma. Hales colloidal iron is often not specific and I do not rely on it, but in the classic case shows diffuse cytoplasmic staining.
Presented by Dr. Jonathan Epstein and prepared by Austin McCuiston.
A 55 year-old man presented with a testicular mass that measured 1.5 cm on ultrasound.
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A 55 year-old man presented with a testicular mass that measured 1.5 cm on ultrasound.
Correct
Answer: Sertoli cell tumor
Histological Description: The lesion is well-circumscribed although not encapsulated. At low magnification there are bands of very eosinophilic collagen dividing tumor. The cells arranged in nests, cords, solid areas with scattered of round to elongated tubules. Cell are polygonal to cuboidal with eosinophilic cytoplasm with focal areas containing clear vacuoles. Nuclear features are bland with central small nucleoli and a virtual absence of mitotic figures.
Discussion: This case is typical for a benign Sertoli cell tumor. One of the most characteristic feature of these lesions is the bands of very eosinophilic collagen dividing the tumor, which is not seen in any of the other entities in the differential diagnosis. In contrast to the “salt and pepper” speckled chromatin seen in carcinoid tumors, Sertoli cell tumors have a central small nucleolus, just as in normal Sertoli cells. The presence of clear vacuoles, when present, is also a good diagnostic clue for a sex cord stromal tumor, as it indicates lipid production. Immunohistochemically, Sertoli cell tumors are variably focally positive for cytokeratin AE1/AE3, usually positive for inhibin alpha, steroid factor 1 (SF1), and calretinin. Synaptophysin can be positive in almost half of the cases with higher rate of chromogranin staining, which can mimic carcinoid tumor. In contrast, a carcinoid tumor and other carcinomas would typically be diffusely positive for cytokeratin AE1/AE3, and negative for inhibin alpha and calretinin. Benign Sertoli cell tumors are typically small (<5 cm), lack atypia, rare mitoses, lack necrosis and vascular invasion. Some experts prefer not to definitively diagnose Sertoli cell tumors as either benign or malignant but rather classify them as having a favorable or unfavorable prognosis based on their histology. In my practice, if a Sertoli cell tumor lacks any atypical feature, I definitively diagnose them as “Benign Sertoli cell tumor” so as to allay any concerns for patients. In cases with only 1 or 2 atypical features, but otherwise benign, I use the term “Sertoli cell tumor with atypical features” and say the prognosis is uncertain. Most malignant Sertoli cell tumors have multiple malignant features.
Incorrect
Answer: Sertoli cell tumor
Histological Description: The lesion is well-circumscribed although not encapsulated. At low magnification there are bands of very eosinophilic collagen dividing tumor. The cells arranged in nests, cords, solid areas with scattered of round to elongated tubules. Cell are polygonal to cuboidal with eosinophilic cytoplasm with focal areas containing clear vacuoles. Nuclear features are bland with central small nucleoli and a virtual absence of mitotic figures.
Discussion: This case is typical for a benign Sertoli cell tumor. One of the most characteristic feature of these lesions is the bands of very eosinophilic collagen dividing the tumor, which is not seen in any of the other entities in the differential diagnosis. In contrast to the “salt and pepper” speckled chromatin seen in carcinoid tumors, Sertoli cell tumors have a central small nucleolus, just as in normal Sertoli cells. The presence of clear vacuoles, when present, is also a good diagnostic clue for a sex cord stromal tumor, as it indicates lipid production. Immunohistochemically, Sertoli cell tumors are variably focally positive for cytokeratin AE1/AE3, usually positive for inhibin alpha, steroid factor 1 (SF1), and calretinin. Synaptophysin can be positive in almost half of the cases with higher rate of chromogranin staining, which can mimic carcinoid tumor. In contrast, a carcinoid tumor and other carcinomas would typically be diffusely positive for cytokeratin AE1/AE3, and negative for inhibin alpha and calretinin. Benign Sertoli cell tumors are typically small (<5 cm), lack atypia, rare mitoses, lack necrosis and vascular invasion. Some experts prefer not to definitively diagnose Sertoli cell tumors as either benign or malignant but rather classify them as having a favorable or unfavorable prognosis based on their histology. In my practice, if a Sertoli cell tumor lacks any atypical feature, I definitively diagnose them as “Benign Sertoli cell tumor” so as to allay any concerns for patients. In cases with only 1 or 2 atypical features, but otherwise benign, I use the term “Sertoli cell tumor with atypical features” and say the prognosis is uncertain. Most malignant Sertoli cell tumors have multiple malignant features.
Presented by Dr. Elizabeth Thompson and prepared by Austin McCuiston.
This is a brain mass in a 45 year old man.
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This is a brain mass in a 45 year old man.
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Answer: Hemangioblastoma
Histology: The tumor is a highly vascular lesion comprised of multiple variably-sized, thin-walled, closely packed/anastomosing vessels with intervening large neoplastic stromal cells with pink-clear foamy cytoplasm with fine vacuoles. The tumor cell nuclei are hyperchromatic and occasionally large and atypical. The neoplastic stromal cells labeled with inhibin and were negative for keratins and EMA. The endothelial cells of the vessels labeled with CD31, CD34 and FXIIIA. An Oil red O stain performed at the time of frozen section was positive, highlighting the lipid content of the neoplastic cells.
Discussion: Hemangioblastomas are well-circumscribed, highly vascular and lipid-rich low-grade neoplasms classically arising in the cerebellum. 10-20% of cases occur in von Hippel-Lindau patients (germline mutation in VHL gene (3p25-26)); most cases are associated with sporadic mutations of the VHL gene. The differential diagnosis is with metastatic carcinoma, particularly renal cell carcinoma, other tumors with clear cell features (neuroendocrine, etc), capillary angioma, meningioma, particularly the microcystic variant and other neural tumors (pilocytic astrocytoma, “diffuse” astrocytoma, and ependymoma. The differential with RCC is potentially difficult as VHL patients are also prone to developing RCC, however RCC would label for keratin and PAX8 and are typically inhibin negative. Capillary angiomas would lack the large interstitial cells with foamy cytoplasm and would stain only for vascular markers but would be inhibin negative with no lipid. Meningiomas would be inhibin negative and EMA and PR positive. Pilocytic astrocytomas can have a microcystic component but would be inhibin negative, as would “diffuse” astrocytomas. Ependymomas would show fewer vessels, perivascular pseudorosettes and would be GFAP and EMA positive.
Incorrect
Answer: Hemangioblastoma
Histology: The tumor is a highly vascular lesion comprised of multiple variably-sized, thin-walled, closely packed/anastomosing vessels with intervening large neoplastic stromal cells with pink-clear foamy cytoplasm with fine vacuoles. The tumor cell nuclei are hyperchromatic and occasionally large and atypical. The neoplastic stromal cells labeled with inhibin and were negative for keratins and EMA. The endothelial cells of the vessels labeled with CD31, CD34 and FXIIIA. An Oil red O stain performed at the time of frozen section was positive, highlighting the lipid content of the neoplastic cells.
Discussion: Hemangioblastomas are well-circumscribed, highly vascular and lipid-rich low-grade neoplasms classically arising in the cerebellum. 10-20% of cases occur in von Hippel-Lindau patients (germline mutation in VHL gene (3p25-26)); most cases are associated with sporadic mutations of the VHL gene. The differential diagnosis is with metastatic carcinoma, particularly renal cell carcinoma, other tumors with clear cell features (neuroendocrine, etc), capillary angioma, meningioma, particularly the microcystic variant and other neural tumors (pilocytic astrocytoma, “diffuse” astrocytoma, and ependymoma. The differential with RCC is potentially difficult as VHL patients are also prone to developing RCC, however RCC would label for keratin and PAX8 and are typically inhibin negative. Capillary angiomas would lack the large interstitial cells with foamy cytoplasm and would stain only for vascular markers but would be inhibin negative with no lipid. Meningiomas would be inhibin negative and EMA and PR positive. Pilocytic astrocytomas can have a microcystic component but would be inhibin negative, as would “diffuse” astrocytomas. Ependymomas would show fewer vessels, perivascular pseudorosettes and would be GFAP and EMA positive.
Presented by Dr. Elizabeth Thompson and prepared by Austin McCuiston.
This is a pancreatic mass in a 9 year old boy.
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This is a pancreatic mass in a 9 year old boy.
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Answer: Solid-pseudopapillary neoplasm
Histology: The tumor is comprised of poorly cohesive monomorphic cells separated by hyalinized to myxoid stromal bands with thin walled blood vessels. The neoplastic cells have variably eosinophillic to foamy or clear cytoplasm. The nuclei show fine chromatin with grooves or indentations. Mixed growth patterns demonstrating variably solid, pseudopapillary and cystic areas are seen. Hyaline globules, clusters of foamy macrophages and cholesterol clefts are present. While well-demarcated from the normal pancreas, a subtle infiltrative pattern can be appreciated at the periphery. The tumor cells labeled for CD10 with aberrant nuclear labeling for beta-catenin.
Discussion: Solid-pseudopapillary neoplasms (SPN) are low grade malignancies arising in the pancreas, predominantly in young women, although this patient was a young boy. The histogenesis of SPN is unclear. The differential is that of solid cellular neoplasms of the pancreas: acinar cell carcinoma, pancreatoblastoma, and neuroendocrine tumors. Acinar cell carcinomas would be bcl10, trypsin and chymotrypsin positive with prominent nucleoli. Pancreatoblastomas can show immunohistochemical evidence of multiple lines of differentiations (acinar, ductal and/or neuroendocrine) with distinct squamoid nests that show nuclear labeling for beta-catenin; squamoid nests are required for the diagnosis and the combination of acinar differentiation plus squamoid nests is diagnostic. Neuroendocrine tumors would be keratin, synaptophysin and chromogranin positive.
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Answer: Solid-pseudopapillary neoplasm
Histology: The tumor is comprised of poorly cohesive monomorphic cells separated by hyalinized to myxoid stromal bands with thin walled blood vessels. The neoplastic cells have variably eosinophillic to foamy or clear cytoplasm. The nuclei show fine chromatin with grooves or indentations. Mixed growth patterns demonstrating variably solid, pseudopapillary and cystic areas are seen. Hyaline globules, clusters of foamy macrophages and cholesterol clefts are present. While well-demarcated from the normal pancreas, a subtle infiltrative pattern can be appreciated at the periphery. The tumor cells labeled for CD10 with aberrant nuclear labeling for beta-catenin.
Discussion: Solid-pseudopapillary neoplasms (SPN) are low grade malignancies arising in the pancreas, predominantly in young women, although this patient was a young boy. The histogenesis of SPN is unclear. The differential is that of solid cellular neoplasms of the pancreas: acinar cell carcinoma, pancreatoblastoma, and neuroendocrine tumors. Acinar cell carcinomas would be bcl10, trypsin and chymotrypsin positive with prominent nucleoli. Pancreatoblastomas can show immunohistochemical evidence of multiple lines of differentiations (acinar, ductal and/or neuroendocrine) with distinct squamoid nests that show nuclear labeling for beta-catenin; squamoid nests are required for the diagnosis and the combination of acinar differentiation plus squamoid nests is diagnostic. Neuroendocrine tumors would be keratin, synaptophysin and chromogranin positive.
Presented by Dr. Pedram Argani and prepared by Austin McCuiston.
This is a 73 year old male who undergoes a resection for a high grade adenocarcinoma of lung origin. The specimen for review is the bronchial margin.
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This is a 73 year old male who undergoes a resection for a high grade adenocarcinoma of lung origin. The specimen for review is the bronchial margin.
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Answer: Metastatic prostatic adenocarcinoma
Histologic Description: This is a bland tubular proliferation which irregularly infiltrates the peribronchial soft tissue. The bland chromatin of the cells suggest the possibility of a neuroendocrine neoplasm, but the architecture is far more tubular than nested. The cytology is far blander than any typical adenocarcinoma of the lung. The glands permeate the fat irregularly, which would be unusual for peribronchial glands which are lobular in their configuration. On further inquiry, this patient had a history of prostatic adenocarcinoma, and the current glands labeled PSA and NKX3.1, supporting the diagnosis.
Differential Diagnosis: Peribronchial glands would be more lobular in their configuration, and typically centered in the bronchial wall submucosa. Adenocarcinoma of the lung demonstrates more cytologic atypia than the bland glands of the current case. Carcinoid tumor is a serious consideration, given the bland cytology of the cells. Carcinoid tumors may demonstrate predominant tubular architecture; however, they should label for neuroendocrine markers and have more salt and pepper type chromatin. The diffuse labeling for prostatic markers along with the history in this case supports the diagnosis of metastatic prostatic adenocarcinoma.
Incorrect
Answer: Metastatic prostatic adenocarcinoma
Histologic Description: This is a bland tubular proliferation which irregularly infiltrates the peribronchial soft tissue. The bland chromatin of the cells suggest the possibility of a neuroendocrine neoplasm, but the architecture is far more tubular than nested. The cytology is far blander than any typical adenocarcinoma of the lung. The glands permeate the fat irregularly, which would be unusual for peribronchial glands which are lobular in their configuration. On further inquiry, this patient had a history of prostatic adenocarcinoma, and the current glands labeled PSA and NKX3.1, supporting the diagnosis.
Differential Diagnosis: Peribronchial glands would be more lobular in their configuration, and typically centered in the bronchial wall submucosa. Adenocarcinoma of the lung demonstrates more cytologic atypia than the bland glands of the current case. Carcinoid tumor is a serious consideration, given the bland cytology of the cells. Carcinoid tumors may demonstrate predominant tubular architecture; however, they should label for neuroendocrine markers and have more salt and pepper type chromatin. The diffuse labeling for prostatic markers along with the history in this case supports the diagnosis of metastatic prostatic adenocarcinoma.
Presented by Dr. Pedram Argani and prepared by Austin McCuiston
This is a 68 year old male with a cystic renal mass.
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This is a 68 year old male with a cystic renal mass.
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Answer: Multilocular cystic renal neoplasm of low malignant potential
Histologic Description: This is a purely cystic renal lesion, and the cysts mold the septa. The septa are thin and without nodules. While most of the septa are characterized by fibrous tissue with foci of ossification, one can appreciate bland clear cells within the septa, frequently associated with prominent capillary vasculature. These cells label for EMA and carbonic anhydrase 9, and have the phenotype of clear cell carcinoma. Because they fail to form nodules, this lesion is regarded as a multilocular cystic renal neoplasm of low malignant potential. Such cases have not demonstrated metastatic behavior in clinical follow-up.
Discussion: Differential Diagnosis: Renal cell carcinomas of clear cell type frequently become cystic; however, in these cases, the septa are thickened and nodular and mold the cysts. Cystic nephroma/mixed epithelial stromal tumor tends to have a wavier, “ovarian-like” stroma that frequently labels for inhibin, and only infrequently has clear cells lining the cysts. Clear cells are typically not present in the septa of cystic nephroma/MEST. The latter occurs far more frequently in adult women. Clear cell papillary renal cell carcinoma is frequently cystic; however, the clear cells line the cysts, and are characterized by prominent subnuclear vacuoles not seen in the current case.
Incorrect
Answer: Multilocular cystic renal neoplasm of low malignant potential
Histologic Description: This is a purely cystic renal lesion, and the cysts mold the septa. The septa are thin and without nodules. While most of the septa are characterized by fibrous tissue with foci of ossification, one can appreciate bland clear cells within the septa, frequently associated with prominent capillary vasculature. These cells label for EMA and carbonic anhydrase 9, and have the phenotype of clear cell carcinoma. Because they fail to form nodules, this lesion is regarded as a multilocular cystic renal neoplasm of low malignant potential. Such cases have not demonstrated metastatic behavior in clinical follow-up.
Discussion: Differential Diagnosis: Renal cell carcinomas of clear cell type frequently become cystic; however, in these cases, the septa are thickened and nodular and mold the cysts. Cystic nephroma/mixed epithelial stromal tumor tends to have a wavier, “ovarian-like” stroma that frequently labels for inhibin, and only infrequently has clear cells lining the cysts. Clear cells are typically not present in the septa of cystic nephroma/MEST. The latter occurs far more frequently in adult women. Clear cell papillary renal cell carcinoma is frequently cystic; however, the clear cells line the cysts, and are characterized by prominent subnuclear vacuoles not seen in the current case.
Presented by Dr. Pedram Argani and prepared by Austin McCuiston
This is 36 year old female with a prior history of malignancy and lung mass.
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This is 36 year old female with a prior history of malignancy and lung mass.
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Histologic Description: This neoplasm consists of nests of polygonal cells with clear to eosinophilic cytoplasm and nuclei with prominent nucleoli. The cells are somewhat discohesive within the nests, leading to an alveolar pattern. The cytoplasm is variably granular. The neoplasm was diffusely and strongly immunoreactive for TFE3. This patient previously had an alveolar soft part sarcoma of the thigh, 15 years prior to this surgery.
Differential Diagnosis: The other neoplasms listed in the differential diagnosis all can have an alveolar pattern. Renal cell carcinoma would be expected to label for PAX8 and not TFE3 unless it were Xp11 translocation carcinoma. The current neoplasm was negative for PAX8. Paraganglioma would label for neuroendocrine markers like chromogranin and synaptophysin, but not for TFE3. Granular cell tumors may show weak labeling for TFE3 but do not demonstrate TFE3 gene rearrangements, are diffusely reactive for S100 protein, and frequently label for inhibin. Only a minority of granular cell tumors demonstrate malignant behavior, whereas alveolar soft part sarcoma frequently metastasizes years after the diagnosis.
Incorrect
Histologic Description: This neoplasm consists of nests of polygonal cells with clear to eosinophilic cytoplasm and nuclei with prominent nucleoli. The cells are somewhat discohesive within the nests, leading to an alveolar pattern. The cytoplasm is variably granular. The neoplasm was diffusely and strongly immunoreactive for TFE3. This patient previously had an alveolar soft part sarcoma of the thigh, 15 years prior to this surgery.
Differential Diagnosis: The other neoplasms listed in the differential diagnosis all can have an alveolar pattern. Renal cell carcinoma would be expected to label for PAX8 and not TFE3 unless it were Xp11 translocation carcinoma. The current neoplasm was negative for PAX8. Paraganglioma would label for neuroendocrine markers like chromogranin and synaptophysin, but not for TFE3. Granular cell tumors may show weak labeling for TFE3 but do not demonstrate TFE3 gene rearrangements, are diffusely reactive for S100 protein, and frequently label for inhibin. Only a minority of granular cell tumors demonstrate malignant behavior, whereas alveolar soft part sarcoma frequently metastasizes years after the diagnosis.
Presented by Dr. Justin Bishop and prepared by Austin McCuiston
70 year old man with leukoplakia of his true vocal cord.
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70 year old man with leukoplakia of his true vocal cord.
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Histology: This biopsy demonstrates numerous fragments of a verrucoid squamous proliferation. The fragments demonstrate varying degrees of cytologic and architectural atypia that warrant a diagnosis of squamous dysplasia. Full-thickness atypia is not seen, however. Invasion is not seen, however, the epithelial-subepithelial interface is not well represented in this biopsy.
Discussion: Verrucoid lesions of the mucosal head and neck are very challenging. Verrucous carcinoma is distinguished from verruca vulgaris/verrucous hyperplasia based on the presence of pushing invasion – which is often difficult to demonstrate in a biopsy. Verrucous carcinoma, verruca vulgaris, and verrucous hyperplasia are all bland at the cytologic level – the lesion depicted here demonstrates too much atypia those diagnoses. On the other hand, it lacks the full-thickness squamous atypia that would be seen in papillary variant of squamous cell carcinoma. Instead, this appears to represent a squamous dysplasia with a verrucoid growth pattern. It is worth noting that this could very well represent an under-sampled well-differentiated squamous cell carcinoma. Because of the exophytic nature of this lesion, the biopsy is relatively superficial and the stromal-epithelial interface is not well represented for evaluating invasion.
Incorrect
Histology: This biopsy demonstrates numerous fragments of a verrucoid squamous proliferation. The fragments demonstrate varying degrees of cytologic and architectural atypia that warrant a diagnosis of squamous dysplasia. Full-thickness atypia is not seen, however. Invasion is not seen, however, the epithelial-subepithelial interface is not well represented in this biopsy.
Discussion: Verrucoid lesions of the mucosal head and neck are very challenging. Verrucous carcinoma is distinguished from verruca vulgaris/verrucous hyperplasia based on the presence of pushing invasion – which is often difficult to demonstrate in a biopsy. Verrucous carcinoma, verruca vulgaris, and verrucous hyperplasia are all bland at the cytologic level – the lesion depicted here demonstrates too much atypia those diagnoses. On the other hand, it lacks the full-thickness squamous atypia that would be seen in papillary variant of squamous cell carcinoma. Instead, this appears to represent a squamous dysplasia with a verrucoid growth pattern. It is worth noting that this could very well represent an under-sampled well-differentiated squamous cell carcinoma. Because of the exophytic nature of this lesion, the biopsy is relatively superficial and the stromal-epithelial interface is not well represented for evaluating invasion.
Presented by Dr. Justin Bishop and prepared by Austin McCuiston
50 year old woman with a thyroid nodule.
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50 year old woman with a thyroid nodule.
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Histology: The background thyroid gland exhibits Hashimoto thyroiditis. There is an ill-defined, unencapsulated tumor consisting of nests and trabeculae of squamoid cells forming duct-like structures. There is a tumor-associated inflammatory cell infiltrate that has a conspicuous number of eosinophils. The tumor cells are relatively bland.
Discussion: Sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE) is a rare form of thyroid carcinoma that typically arises in middle aged women, almost always in the setting of Hashimoto thyroiditis. It consists of a mass-forming proliferation of nests, sheets, cysts, or cords of squamoid cells similar to intermediate cells of salivary mucoepidermoid carcinoma. Overt squamous differentiation, ducts, and mucinous cells may be present, but are not required. The tumor cells exhibit only mild to moderate nuclear pleomorphism, and mitotic rates are low. In addition, there is a sclerotic stromal reaction with an infiltrate of eosinophils. It can be difficult to distinguish from foci of squamous metaplasia commonly seen in Hashimoto thyroiditis – in fact, SMECE may arise from these foci. In contrast to metaplasia, however, SMECE forms a distinct tumor mass. The prognosis of SMECE is generally good; it was originally reported to be extremely indolent, but more recently some aggressive cases have been reported.
Incorrect
Histology: The background thyroid gland exhibits Hashimoto thyroiditis. There is an ill-defined, unencapsulated tumor consisting of nests and trabeculae of squamoid cells forming duct-like structures. There is a tumor-associated inflammatory cell infiltrate that has a conspicuous number of eosinophils. The tumor cells are relatively bland.
Discussion: Sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE) is a rare form of thyroid carcinoma that typically arises in middle aged women, almost always in the setting of Hashimoto thyroiditis. It consists of a mass-forming proliferation of nests, sheets, cysts, or cords of squamoid cells similar to intermediate cells of salivary mucoepidermoid carcinoma. Overt squamous differentiation, ducts, and mucinous cells may be present, but are not required. The tumor cells exhibit only mild to moderate nuclear pleomorphism, and mitotic rates are low. In addition, there is a sclerotic stromal reaction with an infiltrate of eosinophils. It can be difficult to distinguish from foci of squamous metaplasia commonly seen in Hashimoto thyroiditis – in fact, SMECE may arise from these foci. In contrast to metaplasia, however, SMECE forms a distinct tumor mass. The prognosis of SMECE is generally good; it was originally reported to be extremely indolent, but more recently some aggressive cases have been reported.
Presented by Dr. Justin Bishop and prepared by Austin McCuiston
70 year old man with a tongue mass.
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70 year old man with a tongue mass.
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Histology: This tongue mass is a polypoid proliferation of atypical spindled cells. The mucosa overlying the tumor is ulcerated, but the adjacent squamous mucosa exhibits verrucoid dysplastic changes. The spindled cells are overtly malignant with nuclear pleomorphism, an elevated mitotic rate, and atypical mitoses. Immunostains showed that the tumor was negative for p40, AE1/AE3, and Cam5.2.
Discussion: In the mucosal head and neck, a malignant spindle cell neoplasm should be considered a sarcomatoid carcinoma (spindle cell variant of squamous cell carcinoma) until proven otherwise. Confirming the diagnosis of sarcomatoid carcinoma relies on demonstrating epithelial differentiation. This can be done by immunohistochemistry, but many examples of sarcomatoid carcinoma (like this one) are completely negative for epithelial markers. Confirming epithelial differentiation can also be done by finding areas of conventional squamous cell carcinoma within the tumor, or areas of dysplasia/carcinoma-in-situ of the adjacent, intact epithelium. Despite their poorly differentiated appearances, sarcomatoid carcinomas actually tend to have relatively good prognoses; this is likely because they grow in an exophytic fashion and therefore tend to be low-stage.
Incorrect
Histology: This tongue mass is a polypoid proliferation of atypical spindled cells. The mucosa overlying the tumor is ulcerated, but the adjacent squamous mucosa exhibits verrucoid dysplastic changes. The spindled cells are overtly malignant with nuclear pleomorphism, an elevated mitotic rate, and atypical mitoses. Immunostains showed that the tumor was negative for p40, AE1/AE3, and Cam5.2.
Discussion: In the mucosal head and neck, a malignant spindle cell neoplasm should be considered a sarcomatoid carcinoma (spindle cell variant of squamous cell carcinoma) until proven otherwise. Confirming the diagnosis of sarcomatoid carcinoma relies on demonstrating epithelial differentiation. This can be done by immunohistochemistry, but many examples of sarcomatoid carcinoma (like this one) are completely negative for epithelial markers. Confirming epithelial differentiation can also be done by finding areas of conventional squamous cell carcinoma within the tumor, or areas of dysplasia/carcinoma-in-situ of the adjacent, intact epithelium. Despite their poorly differentiated appearances, sarcomatoid carcinomas actually tend to have relatively good prognoses; this is likely because they grow in an exophytic fashion and therefore tend to be low-stage.
Presented by Dr. P. Argani and prepared by Dr. A. McCuiston.
Case 3: This is a 34 year old male with an arm mass.
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This is a 34 year old male with an arm mass.
AE1/AE3
CD34
INI-1
Correct
Answer: Epithelioid sarcoma
Histology: This is a multinodular proliferation of epithelioid cells within the dermis and subcutaneous tissue. Focally, the nodules form tight rounded clusters and are associated with central necrosis, simulating a necrotizing granuloma. However, the lesion does demonstrate greater cytologic atypia than would be expected for benign histiocytes, including rhabdoid cytology in several foci. Moreover, mitotic activity is also evident. The lesion is diffusely immunoreactive for cytokeratin and CD34, and demonstrates loss of INI1 protein by immunohistochemistry, supporting the diagnosis of epithelioid sarcoma.
Differential Diagnosis: Epithelioid sarcoma is often mistaken for a benign granulomatous process such as deep granuloma annulare or infectious granulomas. The greater cytologic atypia is a clue to the diagnosis, which can be supported by diffuse cytokeratin immunoreactivity that would not be identified in a benign granulomatous process. Epithelioid hemangioendothelioma also contains epithelioid neoplastic cells that label CD34 and cytokeratin. However, most of the neoplastic cells would demonstrate vacuolated, “blistered” cytoplasm and the stroma would have a more prominent myxoid appearance. Epithelioid hemangioendothelioma would also label vascular markers like ERG and CD31.
Incorrect
Answer: Epithelioid sarcoma
Histology: This is a multinodular proliferation of epithelioid cells within the dermis and subcutaneous tissue. Focally, the nodules form tight rounded clusters and are associated with central necrosis, simulating a necrotizing granuloma. However, the lesion does demonstrate greater cytologic atypia than would be expected for benign histiocytes, including rhabdoid cytology in several foci. Moreover, mitotic activity is also evident. The lesion is diffusely immunoreactive for cytokeratin and CD34, and demonstrates loss of INI1 protein by immunohistochemistry, supporting the diagnosis of epithelioid sarcoma.
Differential Diagnosis: Epithelioid sarcoma is often mistaken for a benign granulomatous process such as deep granuloma annulare or infectious granulomas. The greater cytologic atypia is a clue to the diagnosis, which can be supported by diffuse cytokeratin immunoreactivity that would not be identified in a benign granulomatous process. Epithelioid hemangioendothelioma also contains epithelioid neoplastic cells that label CD34 and cytokeratin. However, most of the neoplastic cells would demonstrate vacuolated, “blistered” cytoplasm and the stroma would have a more prominent myxoid appearance. Epithelioid hemangioendothelioma would also label vascular markers like ERG and CD31.
Presented by Dr. P. Argani and prepared by Dr. A. McCuiston.
Case 2: This is a 45 year old female with a supraclavicular mass.
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This is a 45 year old female with a supraclavicular mass.
Correct
Answer: Clear cell hidradenoma
Histology: This is a nested and papillary neoplasm which is composed predominantly of bland epithelioid cells with clear cytoplasm. Focally, one can appreciate a second cell layer which resembles umbrella cells draped on the top of the clear cells. The lesion is associated with extensive cystic change, and essentially no mitoses or cytologic atypia is appreciated. This patient had no prior history of malignancy. The lesion was diffusely immunoreactive for p63 and p40. These results support the diagnosis clear cell hidradenoma.
Differential Diagnosis: Clear cell hidradenoma cytologically resembles benign urothelium. Metastatic urothelial carcinoma can be excluded by both history and the absence of cytologic atypia or mitotic activity in the current lesion. The areas of clear cells and papillary architecture raised the differential diagnosis of Xp11 translocation renal cell carcinoma, and the supraclavicular area would be a good site for metastasis from an abdominal primary. However, the urothelial like morphology and diffuse labeling for p40 and p63, along with the absence of a renal mass, argues against Xp11 translocation renal cell carcinoma. Thymoma may occasionally metastasize, and are characterized by bland neoplastic epithelial cells with often squamoid morphology, similar to what is seen in the current case. Thymomas also may be extensive cystic. However, thymoma would be associated immature lymphocytes which would label for TdT, and clinically would be associated with mediastinal mass.
Incorrect
Answer: Clear cell hidradenoma
Histology: This is a nested and papillary neoplasm which is composed predominantly of bland epithelioid cells with clear cytoplasm. Focally, one can appreciate a second cell layer which resembles umbrella cells draped on the top of the clear cells. The lesion is associated with extensive cystic change, and essentially no mitoses or cytologic atypia is appreciated. This patient had no prior history of malignancy. The lesion was diffusely immunoreactive for p63 and p40. These results support the diagnosis clear cell hidradenoma.
Differential Diagnosis: Clear cell hidradenoma cytologically resembles benign urothelium. Metastatic urothelial carcinoma can be excluded by both history and the absence of cytologic atypia or mitotic activity in the current lesion. The areas of clear cells and papillary architecture raised the differential diagnosis of Xp11 translocation renal cell carcinoma, and the supraclavicular area would be a good site for metastasis from an abdominal primary. However, the urothelial like morphology and diffuse labeling for p40 and p63, along with the absence of a renal mass, argues against Xp11 translocation renal cell carcinoma. Thymoma may occasionally metastasize, and are characterized by bland neoplastic epithelial cells with often squamoid morphology, similar to what is seen in the current case. Thymomas also may be extensive cystic. However, thymoma would be associated immature lymphocytes which would label for TdT, and clinically would be associated with mediastinal mass.
Presented by Dr. P. Argani and prepared by Dr. A. McCuiston.
Case 1: This is a 54 year old male with multiple pleural masses.
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This is a 54 year old male with multiple pleural masses.
BCL-2
Correct
Answer: Synovial sarcoma
Histology: This is a highly cellular but non-pleomorphic spindle cell neoplasm. Spindle cells are arranged in short fascicles, and focally there is cystic change within the tumor. The cells have variably prominent nucleoli, and have a plump spindle cell shape. The cytoplasm is minimal and largely clear. By immunohistochemistry, the tumor is immunoreactive for BCL2 but not for CD34. Molecular analysis revealed evidence of the SYT-SSX 2 gene fusion, supporting the diagnosis of synovial sarcoma.
Differential Diagnosis: Solitary fibrous tumor would be typically less cellular and associated with more collagen production. Malignant solitary fibrous tumors exist; however, these lesions would typically label for CD34 and would not have the SYT-SSX gene fusion of synovial sarcoma. Sarcomatoid malignant mesothelioma is typically more pleomorphic, and would demonstrate diffuse immunoreactivity for cytokeratin (unlike the typical focal immunoreactivity for cytokeratin in synovial sarcoma of spindle cell type). Leiomyosarcoma would typically demonstrate more pleomorphism, would have more eosinophilic cytoplasm, and would label desmin.
Synovial sarcoma may present in the pleura as a primary neoplasm in this site, though metastasis should be excluded clinically.
Incorrect
Answer: Synovial sarcoma
Histology: This is a highly cellular but non-pleomorphic spindle cell neoplasm. Spindle cells are arranged in short fascicles, and focally there is cystic change within the tumor. The cells have variably prominent nucleoli, and have a plump spindle cell shape. The cytoplasm is minimal and largely clear. By immunohistochemistry, the tumor is immunoreactive for BCL2 but not for CD34. Molecular analysis revealed evidence of the SYT-SSX 2 gene fusion, supporting the diagnosis of synovial sarcoma.
Differential Diagnosis: Solitary fibrous tumor would be typically less cellular and associated with more collagen production. Malignant solitary fibrous tumors exist; however, these lesions would typically label for CD34 and would not have the SYT-SSX gene fusion of synovial sarcoma. Sarcomatoid malignant mesothelioma is typically more pleomorphic, and would demonstrate diffuse immunoreactivity for cytokeratin (unlike the typical focal immunoreactivity for cytokeratin in synovial sarcoma of spindle cell type). Leiomyosarcoma would typically demonstrate more pleomorphism, would have more eosinophilic cytoplasm, and would label desmin.
Synovial sarcoma may present in the pleura as a primary neoplasm in this site, though metastasis should be excluded clinically.
Presented by Dr. Cimino-Mathews and prepared by Dr. Austin McCuiston.
Clinical history: A 60 year-old male with a parotid mass.
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Clinical history: A 60 year-old male with a parotid mass.
Correct
Answer: Acinic cell carcinoma with high grade transformation
Histology: The resection specimen shows a serous salivary gland, consistent with the parotid gland. In it, there is a lesion comprised predominantly of small to large infiltrating nests of malignant epithelial cells with central comedo-type necrosis. The nests of cells display solid, cribriform and rosette-like architecture. The cells display nuclear atypia with a brisk mitotic rate, and the cytoplasm is primarily eosinophilic with focal blue zymogen granules. Perineural invasion is identified. Adjacent to this high grade component, a lower grade component is identified consisting of cells with bland nuclei, prominent granular cytoplasm, and a microcystic growth pattern.
Discussion: The histology of the low grade component is characteristic of acinic cell carcinoma of the parotid salivary gland, and the presence of the high grade component is consistent with high grade transformation of acinic cell carcinoma. High grade transformation can be seen in otherwise low grade malignant salivary gland neoplasms, which is contrast to malignancy arising from a pre-existing benign tumor as seen in carcinoma ex pleomorphic adenoma. Acinic cell carcinomas of the salivary glands arise from the serous acinar cells, thus most acinic cell carcinomas arise in the parotid gland, which is contains exclusively serous acini. In contrast, the sublingual gland has exclusively mucinous acini, the submandibular gland has mixed serous and mucinous aciniand the minor salivary glands are largely mixed. In fact, many acinic cell carcinomas previously diagnosed in non-parotid salivary glands turn out to be mammary analog secretory carcinomas, which histologically mimic acinic cell carcinomas due to the microcystic architecture but are distinct and in fact harbor fusions in ETV6.
Reference(s):
– Bishop JA, Yonescu R, Batista D, Eisele DW, Westra WH. Most nonparotid “acinic cell carcinomas” represent mammary analog secretory carcinomas. Am J Surg Pathol. 2013 Jul;37(7):1053-7.
– Zarbo RJ. Salivary Gland Neoplasia: A Review for the Practicing Pathologist Mod Pathol 2002;15(3):298–323.
Incorrect
Answer: Acinic cell carcinoma with high grade transformation
Histology: The resection specimen shows a serous salivary gland, consistent with the parotid gland. In it, there is a lesion comprised predominantly of small to large infiltrating nests of malignant epithelial cells with central comedo-type necrosis. The nests of cells display solid, cribriform and rosette-like architecture. The cells display nuclear atypia with a brisk mitotic rate, and the cytoplasm is primarily eosinophilic with focal blue zymogen granules. Perineural invasion is identified. Adjacent to this high grade component, a lower grade component is identified consisting of cells with bland nuclei, prominent granular cytoplasm, and a microcystic growth pattern.
Discussion: The histology of the low grade component is characteristic of acinic cell carcinoma of the parotid salivary gland, and the presence of the high grade component is consistent with high grade transformation of acinic cell carcinoma. High grade transformation can be seen in otherwise low grade malignant salivary gland neoplasms, which is contrast to malignancy arising from a pre-existing benign tumor as seen in carcinoma ex pleomorphic adenoma. Acinic cell carcinomas of the salivary glands arise from the serous acinar cells, thus most acinic cell carcinomas arise in the parotid gland, which is contains exclusively serous acini. In contrast, the sublingual gland has exclusively mucinous acini, the submandibular gland has mixed serous and mucinous aciniand the minor salivary glands are largely mixed. In fact, many acinic cell carcinomas previously diagnosed in non-parotid salivary glands turn out to be mammary analog secretory carcinomas, which histologically mimic acinic cell carcinomas due to the microcystic architecture but are distinct and in fact harbor fusions in ETV6.
Reference(s):
– Bishop JA, Yonescu R, Batista D, Eisele DW, Westra WH. Most nonparotid “acinic cell carcinomas” represent mammary analog secretory carcinomas. Am J Surg Pathol. 2013 Jul;37(7):1053-7.
– Zarbo RJ. Salivary Gland Neoplasia: A Review for the Practicing Pathologist Mod Pathol 2002;15(3):298–323.
Presented by Dr. Cimino-Mathews and prepared by Dr. Austin McCuiston.
Clinical history: A 20 year-old male with a lung mass.
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Clinical history: A 20 year-old male with a lung mass.
Correct
Answer: Metastatic osteosarcoma
Histology: The lung wedge resection contains a fairly well circumscribed, peripheral nodule comprised of mesenchymal cells with an associated mineralized matrix of osteoid, as well as focal cartilaginous differentiation. The individual cells are spindled to epithelioid with focally prominent nucleoli and a low mitotic rate. There is no necrosis. There is no associated fat or respiratory epithelium in the lesion.
Discussion: This is a metastatic chondroblastic osteosarcoma in a patient with a known history of a tibial primary. Osteosarcomas by definition are sarcomas in which the neoplastic cells produce osteoid; chondroblastic osetosarcomas contain islands or lobules of cartilage with atypical chondrocytes. The differential diagnosis of a cartilage-forming lesion in the lung includes hamartoma and chondroma. Pulmonary hamartomas are typically well-circumscribed, peripheral nodules (although they can occur centrally) that are comprised of mature hyaline cartilage, clefts lined by respiratory epithelium, fat and smooth muscle. Chondromas are typically central and arise from the tracheal or bronchial cartilage; they are benign proliferations of chondrocytes and are generally lobulated. Metastatic sarcomatoid (metaplastic) carcinomas can also contain malignant osseous or cartilaginous components, and clinical history is key in this setting. The radiographic characteristics (central vs. peripheral; circumscribed vs. infiltrative; bone/matrix-producing vs. non-matrix producing) should be taken into consideration with any chondroblastic or osteoblastic lesion.
Reference(s):
-Klein MJ, Siegal GP. Osteosarcoma: anatomic and histologic variants. Am J Clin Pathol. 2006 Apr;125(4):555-81.
Incorrect
Answer: Metastatic osteosarcoma
Histology: The lung wedge resection contains a fairly well circumscribed, peripheral nodule comprised of mesenchymal cells with an associated mineralized matrix of osteoid, as well as focal cartilaginous differentiation. The individual cells are spindled to epithelioid with focally prominent nucleoli and a low mitotic rate. There is no necrosis. There is no associated fat or respiratory epithelium in the lesion.
Discussion: This is a metastatic chondroblastic osteosarcoma in a patient with a known history of a tibial primary. Osteosarcomas by definition are sarcomas in which the neoplastic cells produce osteoid; chondroblastic osetosarcomas contain islands or lobules of cartilage with atypical chondrocytes. The differential diagnosis of a cartilage-forming lesion in the lung includes hamartoma and chondroma. Pulmonary hamartomas are typically well-circumscribed, peripheral nodules (although they can occur centrally) that are comprised of mature hyaline cartilage, clefts lined by respiratory epithelium, fat and smooth muscle. Chondromas are typically central and arise from the tracheal or bronchial cartilage; they are benign proliferations of chondrocytes and are generally lobulated. Metastatic sarcomatoid (metaplastic) carcinomas can also contain malignant osseous or cartilaginous components, and clinical history is key in this setting. The radiographic characteristics (central vs. peripheral; circumscribed vs. infiltrative; bone/matrix-producing vs. non-matrix producing) should be taken into consideration with any chondroblastic or osteoblastic lesion.
Reference(s):
-Klein MJ, Siegal GP. Osteosarcoma: anatomic and histologic variants. Am J Clin Pathol. 2006 Apr;125(4):555-81.
Presented by Dr. Cimino-Mathews and prepared by Dr. Austin McCuiston
Clinical history: 30 year-old female with rectal bleeding.
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Clinical history: 30 year-old female with rectal bleeding
Correct
Answer: Primary anorectal melanoma
Histology: The excisional specimen contains a mass centered in the anorectal mucosa that is comprised of atypical spindle cells arranged in nests and intersecting fascicles. The lesional cells contain elongated nuclei with fairly evenly dispersed chromatin, occasional binucleation, occasional intranuclear inclusions, and a brisk mitotic rate. There is no associated in situ component. Immunostains show the lesional cells to be diffusely positive for S100 protein and negative for AE1/AE3.
Discussion: Mucosal melanomas are rare but can occur in mucosal sites throughout the body, including at the anorectal junction. Melanomas of any body site can have varied histologic appearances, ranging from epithelioid “pink cell” tumors with prominent nucleoli to spindle cell tumors, thus the differential diagnosis can be broad and varied depending upon the organ site and the morphology. In the gastrointestinal tract, it is useful to think of the differential diagnosis of spindle cell tumors in the context of the “layer” of the wall. Spindle cell lesions that are centered in the mucosal layer (such as this tumor) include mucosal melanoma, benign fibroblastic polyp/perineurioma, some benign nerve sheath tumors, leiomyomas of the muscularis mucosa, and rarely synovial sarcomas. In contrast, inflammatory fibroid polyps are centered in the submucosa; gastrointestinal stromal tumors (GIST), most schwannomas, leiomyomas, glomus tumors and clear cell sarcomas are centered in the muscularis propria; and inflammatory myofibroblastic tumors, fibromatosis, and sclerosing diseases are centered in the subserosa/mesentery. Metastatic lesions from other organ sites can involve any layer. Recognizing the morphologic features of the lesions and the layer of the gastrointestinal tract involved, along with performing a targeted immunopanel and knowing the clinical history, can enable accurate diagnosis.
Reference(s): (if available, if not, do not include)
– Voltaggio L, Montgomery EA. Gastrointestinal tract spindle cell lesions–just like real estate, it’s all about location. Mod Pathol. 2015 Jan;28 Suppl 1:S47-66.
Incorrect
Answer: Primary anorectal melanoma
Histology: The excisional specimen contains a mass centered in the anorectal mucosa that is comprised of atypical spindle cells arranged in nests and intersecting fascicles. The lesional cells contain elongated nuclei with fairly evenly dispersed chromatin, occasional binucleation, occasional intranuclear inclusions, and a brisk mitotic rate. There is no associated in situ component. Immunostains show the lesional cells to be diffusely positive for S100 protein and negative for AE1/AE3.
Discussion: Mucosal melanomas are rare but can occur in mucosal sites throughout the body, including at the anorectal junction. Melanomas of any body site can have varied histologic appearances, ranging from epithelioid “pink cell” tumors with prominent nucleoli to spindle cell tumors, thus the differential diagnosis can be broad and varied depending upon the organ site and the morphology. In the gastrointestinal tract, it is useful to think of the differential diagnosis of spindle cell tumors in the context of the “layer” of the wall. Spindle cell lesions that are centered in the mucosal layer (such as this tumor) include mucosal melanoma, benign fibroblastic polyp/perineurioma, some benign nerve sheath tumors, leiomyomas of the muscularis mucosa, and rarely synovial sarcomas. In contrast, inflammatory fibroid polyps are centered in the submucosa; gastrointestinal stromal tumors (GIST), most schwannomas, leiomyomas, glomus tumors and clear cell sarcomas are centered in the muscularis propria; and inflammatory myofibroblastic tumors, fibromatosis, and sclerosing diseases are centered in the subserosa/mesentery. Metastatic lesions from other organ sites can involve any layer. Recognizing the morphologic features of the lesions and the layer of the gastrointestinal tract involved, along with performing a targeted immunopanel and knowing the clinical history, can enable accurate diagnosis.
Reference(s): (if available, if not, do not include)
– Voltaggio L, Montgomery EA. Gastrointestinal tract spindle cell lesions–just like real estate, it’s all about location. Mod Pathol. 2015 Jan;28 Suppl 1:S47-66.
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