Presented by Dr. Argani and prepared by J. Stephen Nix
This is a 73 year old male with bilateral adrenal enlargement and a history of an ACTH-producing tumor
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This is a 73 year old male with bilateral adrenal enlargement and a history of an ACTH-producing tumor
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Answer: B
Histologic Description: The background adrenal shows the changes consistent with ACTH-dependent hyperplasia; specifically, there is depletion of the lipid-rich zona fasciculata, and instead there is a prominent zona reticularis characterized by cells with granular eosinophilic cytoplasm and occasional pigment. Admixed within this hyperplasia are small nests of primitive small round cells which have mitotic activity and demonstrate nuclear molding, consistent with metastatic small cell carcinoma. This patient had a history of small cell carcinoma of the lung which was ACTH secreting. The patient underwent adrenalectomy with the thought that the bilateral adrenals would demonstrate only cortical hyperplasia. The small foci of metastatic small cell carcinoma were an unsuspected finding.
Differential Diagnosis: Adrenal cortical hyperplasia does not typically demonstrate a prominent lymphoid reaction. Even if this were present, the primitive chromatin and nuclear molding of the small cell carcinoma is different than that of lymphoid tissue. Medullary hyperplasia would feature nests of epithelioid cells with clear or granular purple cytoplasm, which is different than the cortical hyperplasia seen in this case. Cortical adenoma would be a discrete lesion in a background of normal adrenal. Neither of the latter two diagnoses account for the metastatic small cell carcinoma component of this case.
Incorrect
Answer: B
Histologic Description: The background adrenal shows the changes consistent with ACTH-dependent hyperplasia; specifically, there is depletion of the lipid-rich zona fasciculata, and instead there is a prominent zona reticularis characterized by cells with granular eosinophilic cytoplasm and occasional pigment. Admixed within this hyperplasia are small nests of primitive small round cells which have mitotic activity and demonstrate nuclear molding, consistent with metastatic small cell carcinoma. This patient had a history of small cell carcinoma of the lung which was ACTH secreting. The patient underwent adrenalectomy with the thought that the bilateral adrenals would demonstrate only cortical hyperplasia. The small foci of metastatic small cell carcinoma were an unsuspected finding.
Differential Diagnosis: Adrenal cortical hyperplasia does not typically demonstrate a prominent lymphoid reaction. Even if this were present, the primitive chromatin and nuclear molding of the small cell carcinoma is different than that of lymphoid tissue. Medullary hyperplasia would feature nests of epithelioid cells with clear or granular purple cytoplasm, which is different than the cortical hyperplasia seen in this case. Cortical adenoma would be a discrete lesion in a background of normal adrenal. Neither of the latter two diagnoses account for the metastatic small cell carcinoma component of this case.
Presented by Dr. Argani and prepared by J. Stephen Nix
This is a 6 year old male with 9 cm renal mass
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This is a 6 year old male with 9 cm renal mass
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Answer: A
Histologic Description: This is an encapsulated neoplasm which is epithelial predominant. Most of the neoplasm consists of tubules lined by primitive columnar cells with mitotic activity, diagnostic of epithelial Wilms tumor. Of interest, there are areas at the edge of this encapsulated neoplasm where the cells are better differentiated, more cuboidal, less mitotically active, and associated with sclerotic stroma and psammomatous microcalcifications. These areas resemble metanephric adenoma. It is not uncommon for epithelial Wilms tumors to have areas within them that resemble metanephric adenoma.
Differential Diagnosis: Metanephric adenoma would not have the proliferative primitive areas with columnar nucleoli that are typical of Wilms tumor. Metanephric adenofibroma would feature a spindle cell stroma that is identical to metanephric stromal tumor, along with areas of metanephric adenoma. These lesions also may be associated with papillary renal cell carcinoma (composite metanephric adenofibroma-papillary renal cell carcinoma). The current case lacks the stroma and papillary renal cell carcinoma component of such lesions, and the mitotically active primitive tubules are better for Wilms tumor than metanephric adenoma. Papillary renal cell carcinoma may have a blue cell appearance (so-called type I papillary renal cell carcinoma). However, the nuclei of such lesions are typically more open, and these neoplasms do not label for WT1 like metanephric adenoma and Wilms tumor.
Incorrect
Answer: A
Histologic Description: This is an encapsulated neoplasm which is epithelial predominant. Most of the neoplasm consists of tubules lined by primitive columnar cells with mitotic activity, diagnostic of epithelial Wilms tumor. Of interest, there are areas at the edge of this encapsulated neoplasm where the cells are better differentiated, more cuboidal, less mitotically active, and associated with sclerotic stroma and psammomatous microcalcifications. These areas resemble metanephric adenoma. It is not uncommon for epithelial Wilms tumors to have areas within them that resemble metanephric adenoma.
Differential Diagnosis: Metanephric adenoma would not have the proliferative primitive areas with columnar nucleoli that are typical of Wilms tumor. Metanephric adenofibroma would feature a spindle cell stroma that is identical to metanephric stromal tumor, along with areas of metanephric adenoma. These lesions also may be associated with papillary renal cell carcinoma (composite metanephric adenofibroma-papillary renal cell carcinoma). The current case lacks the stroma and papillary renal cell carcinoma component of such lesions, and the mitotically active primitive tubules are better for Wilms tumor than metanephric adenoma. Papillary renal cell carcinoma may have a blue cell appearance (so-called type I papillary renal cell carcinoma). However, the nuclei of such lesions are typically more open, and these neoplasms do not label for WT1 like metanephric adenoma and Wilms tumor.
Presented by Dr. Argani and prepared by J. Stephen Nix
This is a 71 year old male with an axillary mass
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This is a 71 year old male with an axillary mass
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Answer: A
Histologic Description: The lymph node contains sheets of epithelioid cells with vesicular chromatin, prominent nucleoli, and eccentric pink cytoplasm, give them a “rhabdoid” phenotype. There is abundant mitotic activity and central necrosis. These areas of the neoplasm demonstrated no immunoreactivity for melanocytic markers, but demonstrated patchy immunoreactivity for CD30 and CD138, raising the possibility of anaplastic large cell lymphoma, undifferentiated carcinoma or myeloma. However, at the edge of the lymph node are less pleomorphic though unequivocally malignant cells forming nests rather than sheets. By immunohistochemistry, these cells label for melanocytic markers like melan A, HMB45 and SOX10. Therefore, the best diagnosis is metastatic melanoma. CD30 and CD138 are markers which are notoriously non-specific in the setting of high grade malignancies.
Differentiation Diagnosis: True rhabdoid tumor would be virtually unheard of in this age group, and would demonstrate loss of INI1 protein. Metastatic carcinoma could label for CD138, but should label for cytokeratins and not melanocytic markers. CD30 immunoreactivity is not specific for anaplastic large cell lymphoma, and the melanocytic marker immunoreactivity eliminates this diagnosis.
This case illustrates the importance of identifying a better differentiated component of a neoplasm when one performs immunohistochemistry. Staining an undifferentiated high grade area often reveals nothing.
Incorrect
Answer: A
Histologic Description: The lymph node contains sheets of epithelioid cells with vesicular chromatin, prominent nucleoli, and eccentric pink cytoplasm, give them a “rhabdoid” phenotype. There is abundant mitotic activity and central necrosis. These areas of the neoplasm demonstrated no immunoreactivity for melanocytic markers, but demonstrated patchy immunoreactivity for CD30 and CD138, raising the possibility of anaplastic large cell lymphoma, undifferentiated carcinoma or myeloma. However, at the edge of the lymph node are less pleomorphic though unequivocally malignant cells forming nests rather than sheets. By immunohistochemistry, these cells label for melanocytic markers like melan A, HMB45 and SOX10. Therefore, the best diagnosis is metastatic melanoma. CD30 and CD138 are markers which are notoriously non-specific in the setting of high grade malignancies.
Differentiation Diagnosis: True rhabdoid tumor would be virtually unheard of in this age group, and would demonstrate loss of INI1 protein. Metastatic carcinoma could label for CD138, but should label for cytokeratins and not melanocytic markers. CD30 immunoreactivity is not specific for anaplastic large cell lymphoma, and the melanocytic marker immunoreactivity eliminates this diagnosis.
This case illustrates the importance of identifying a better differentiated component of a neoplasm when one performs immunohistochemistry. Staining an undifferentiated high grade area often reveals nothing.
Presented by Dr. Epstein and prepared by J. Stephen Nix
A 15 year old male was noted to have a paratesticular mass.
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1. Question
A 15 year old male was noted to have a paratesticular mass.
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Answer: C
Histology: The tumor consists of a cellular spindle cell and small blue cell tumor. Focally, one can appreciate some eosinophilic spindling of the cytoplasm. Some cells are anaplastic with large pleomorphic nuclei and markedly atypical mitotic figures.
Discussion: The key issues in this case is the patient’s age and the location of the tumor. It is critical to distinguish intratesticular vs. paratesticular lesions since the differential diagnosis is for the most part totally different. In the paratesticular region, an overtly malignant pleomorphic spindle cell tumor in an adult is almost always a de-differentiated liposarcoma and in a child or young adult, it is a rhabdomyosarcoma. This case, although there are some cells typical of rhabdomyoblasts, has relatively few cells with eosinophilic cytoplasmic tails showing overt skeletal muscle differentiation. Some paratesticular rhabdomyosarcomas will have more cells that resemble skeletal muscle and in some cases one can even see cross striations. The current case is also exceptional for the pronounced pleomorphism and atypical mitotic figures. This case would be noted in the report as having anaplasia in an analogous fashion to Wilms tumor with anaplasia. Although there is scant literature on these cases, they appear to have a worse prognosis. The diagnosis can be confirmed with strong staining for desmin and also some immunoreactivity for myogenin. However, one must recognize that myogenin may label only rare cells.
Incorrect
Answer: C
Histology: The tumor consists of a cellular spindle cell and small blue cell tumor. Focally, one can appreciate some eosinophilic spindling of the cytoplasm. Some cells are anaplastic with large pleomorphic nuclei and markedly atypical mitotic figures.
Discussion: The key issues in this case is the patient’s age and the location of the tumor. It is critical to distinguish intratesticular vs. paratesticular lesions since the differential diagnosis is for the most part totally different. In the paratesticular region, an overtly malignant pleomorphic spindle cell tumor in an adult is almost always a de-differentiated liposarcoma and in a child or young adult, it is a rhabdomyosarcoma. This case, although there are some cells typical of rhabdomyoblasts, has relatively few cells with eosinophilic cytoplasmic tails showing overt skeletal muscle differentiation. Some paratesticular rhabdomyosarcomas will have more cells that resemble skeletal muscle and in some cases one can even see cross striations. The current case is also exceptional for the pronounced pleomorphism and atypical mitotic figures. This case would be noted in the report as having anaplasia in an analogous fashion to Wilms tumor with anaplasia. Although there is scant literature on these cases, they appear to have a worse prognosis. The diagnosis can be confirmed with strong staining for desmin and also some immunoreactivity for myogenin. However, one must recognize that myogenin may label only rare cells.
Presented by Dr. Epstein and prepared by J. Stephen Nix
A 65 year old man presents with hematuria and a papillary tumor in the bladder.
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A 65 year old man presents with hematuria and a papillary tumor in the bladder.
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Answer: B
Histology: The lesions has discrete, delicate, non-fused papillae. Urothelium is overtly thick, even at low magnification. Cytology is normal or at most slightly enlarged nuclei with some cells having nuclear grooves. Umbrella cells are inconspicuous. Mitotic figures are virtually absent or at most rarely seen towards the basement membrane.
Discussion: In contrast to a benign urothelial papilloma, the thickness is overtly thicker than normal urothelium. In contrast to a low grade papillary urothelial carcinoma, there is a complete lack of cytological atypia with field after field showing monotonous bland urothelial cells without larger hyperchromatic nuclei. Also mitotic figures are virtually absent. Although PUNLMPS have about a 30% risk of recurrence over 15 years, typically as PUNLMP, only rare cases recur as higher grade cancer with invasion. In contrast to low grade papillary urothelial carcinoma where uncommonly there may be associated invasive low grade urothelial carcinoma, there is never concurrent invasion with a PUNLMP. Also no one with a PUNLMP will have a death due to bladder cancer. Although patients with PUNLMP will still be followed by urologists like a low grade papillary urothelial carcinoma, the patient is not labeled with a “carcinoma” diagnosis.
Incorrect
Answer: B
Histology: The lesions has discrete, delicate, non-fused papillae. Urothelium is overtly thick, even at low magnification. Cytology is normal or at most slightly enlarged nuclei with some cells having nuclear grooves. Umbrella cells are inconspicuous. Mitotic figures are virtually absent or at most rarely seen towards the basement membrane.
Discussion: In contrast to a benign urothelial papilloma, the thickness is overtly thicker than normal urothelium. In contrast to a low grade papillary urothelial carcinoma, there is a complete lack of cytological atypia with field after field showing monotonous bland urothelial cells without larger hyperchromatic nuclei. Also mitotic figures are virtually absent. Although PUNLMPS have about a 30% risk of recurrence over 15 years, typically as PUNLMP, only rare cases recur as higher grade cancer with invasion. In contrast to low grade papillary urothelial carcinoma where uncommonly there may be associated invasive low grade urothelial carcinoma, there is never concurrent invasion with a PUNLMP. Also no one with a PUNLMP will have a death due to bladder cancer. Although patients with PUNLMP will still be followed by urologists like a low grade papillary urothelial carcinoma, the patient is not labeled with a “carcinoma” diagnosis.
Presented by Dr. Epstein and prepared by J. Stephen Nix
A 45 year old male presented with a 3 cm. testicular mass.
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A 45 year old male presented with a 3 cm. testicular mass.
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Answer: B
Histology: The lesion consists of a fairly well-circumscribed but unencapsulated mass with intersecting bands of eosinophilic collagen. There is minimal entrapped seminiferous tubules seen at the periphery of the nodule. The surrounding interstitium shows normal Leydig cells. The tumor sheets out without tubular formation. The neoplastic cells are polygonal in shape with fairly abundant lightly eosinophilic cytoplasm, which in areas at the edge of the tumor show vacuolization. Tumor cell nuclei are round with small but conspicuous nucleoli.
Description: This case has some features that make it a not so straightforward to diagnose. The presence of bands of eosinophilic collagen is more characteristic of a Sertoli cell tumor. Also the cytoplasm is not a bright eosinophilic as the normal Leydig cells in the surrounding testis. However, the sheeting out of tumor without tubule formation is typical of Leydig as opposed to Sertoli cell tumor. Also the presence of clear vacuoles containing lipid seen in steroid secreting Ctumor is characteristic of Leydig cell tumor. The tumor lacks any malignant features as it is small, lacks increased mitoses, lacks necrosis, does not widely infiltrate into the normal testis, and does have necrosis or pleomorphism. If one was not sure if the tumor was a sec cord stromal tumor, the best IHC markers would be SF-1 and if not available inhibin. In contrast to Leydig cell hyperplasia, the surrounding testis does not show multinodular collections of Leydig cells and with Leydig cell tumors the surrounding Leydig cells are either normal or decreased in quantity.
Incorrect
Answer: B
Histology: The lesion consists of a fairly well-circumscribed but unencapsulated mass with intersecting bands of eosinophilic collagen. There is minimal entrapped seminiferous tubules seen at the periphery of the nodule. The surrounding interstitium shows normal Leydig cells. The tumor sheets out without tubular formation. The neoplastic cells are polygonal in shape with fairly abundant lightly eosinophilic cytoplasm, which in areas at the edge of the tumor show vacuolization. Tumor cell nuclei are round with small but conspicuous nucleoli.
Description: This case has some features that make it a not so straightforward to diagnose. The presence of bands of eosinophilic collagen is more characteristic of a Sertoli cell tumor. Also the cytoplasm is not a bright eosinophilic as the normal Leydig cells in the surrounding testis. However, the sheeting out of tumor without tubule formation is typical of Leydig as opposed to Sertoli cell tumor. Also the presence of clear vacuoles containing lipid seen in steroid secreting Ctumor is characteristic of Leydig cell tumor. The tumor lacks any malignant features as it is small, lacks increased mitoses, lacks necrosis, does not widely infiltrate into the normal testis, and does have necrosis or pleomorphism. If one was not sure if the tumor was a sec cord stromal tumor, the best IHC markers would be SF-1 and if not available inhibin. In contrast to Leydig cell hyperplasia, the surrounding testis does not show multinodular collections of Leydig cells and with Leydig cell tumors the surrounding Leydig cells are either normal or decreased in quantity.
Presented by Dr. Argani and prepared by J. Stephen Nix
This is an 87 year old female with a 1 cm breast mass.
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This is an 87 year old female with a 1 cm breast mass.
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Answer: C
Histologic Description: This is a fairly well delineated fibroblastic nodule with extensive hyalinization. There is subtle infiltration at the edge of the lesion, and abrupt variations in cellularity, which raise suspicion for metaplastic carcinoma. The spindle cells are diffusely immunoreactive for high molecular weight cytokeratin (34BE12), supporting the diagnosis of spindle cell metaplastic carcinoma.
Differential Diagnosis: Fibroadenoma would not label for high molecular weight cytokeratin, and typically labels for CD34. Sclerosed papillomas often have a hyalinized rounded appearance, but would not demonstrate high molecular weight cytokeratin labeling. Duct ectasia is characterized by markedly dilated ducts associated with squamous metaplasia and periductal chronic inflammation and histiocytes, not seen in the current case.
This case illustrates a subtle low grade spindle cell metaplastic carcinoma. Clues to the diagnosis include abrupt transitions in cellularity within the lesion, and the layered, plaque like hyaline fibrosis within the lesion.
Incorrect
Answer: C
Histologic Description: This is a fairly well delineated fibroblastic nodule with extensive hyalinization. There is subtle infiltration at the edge of the lesion, and abrupt variations in cellularity, which raise suspicion for metaplastic carcinoma. The spindle cells are diffusely immunoreactive for high molecular weight cytokeratin (34BE12), supporting the diagnosis of spindle cell metaplastic carcinoma.
Differential Diagnosis: Fibroadenoma would not label for high molecular weight cytokeratin, and typically labels for CD34. Sclerosed papillomas often have a hyalinized rounded appearance, but would not demonstrate high molecular weight cytokeratin labeling. Duct ectasia is characterized by markedly dilated ducts associated with squamous metaplasia and periductal chronic inflammation and histiocytes, not seen in the current case.
This case illustrates a subtle low grade spindle cell metaplastic carcinoma. Clues to the diagnosis include abrupt transitions in cellularity within the lesion, and the layered, plaque like hyaline fibrosis within the lesion.
Presented by Dr. Argani and prepared by J. Stephen Nix
This is a 32 year old male with a paraspinal mass.
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This is a 32 year old male with a paraspinal mass.
Correct
Answer: D
Histologic Description: This is a highly cellular spindle cell neoplasm, with occasional mitotic figures. The neoplasm permeates bone, which suggests an aggressive process. The neoplasm is diffusely immunoreactive for S100 protein, and other areas demonstrates the hyalinized vessels and lower cellularity typical of schwannoma.
Differential Diagnosis: Rhabdomyosarcoma would label for myogenin and desmin, it would have more eosinophilic cytoplasm. Leiomyosarcoma would demonstrate more eosinophilic cytoplasm and label for desmin. Malignant peripheral nerve sheath tumors are typically not diffusely S100 positive like schwannoma, and demonstrate greater atypia and mitotic activity.
Cellular schwannomas may erode bone and demonstrate mitotic activity, mimicking a malignancy.
Incorrect
Answer: D
Histologic Description: This is a highly cellular spindle cell neoplasm, with occasional mitotic figures. The neoplasm permeates bone, which suggests an aggressive process. The neoplasm is diffusely immunoreactive for S100 protein, and other areas demonstrates the hyalinized vessels and lower cellularity typical of schwannoma.
Differential Diagnosis: Rhabdomyosarcoma would label for myogenin and desmin, it would have more eosinophilic cytoplasm. Leiomyosarcoma would demonstrate more eosinophilic cytoplasm and label for desmin. Malignant peripheral nerve sheath tumors are typically not diffusely S100 positive like schwannoma, and demonstrate greater atypia and mitotic activity.
Cellular schwannomas may erode bone and demonstrate mitotic activity, mimicking a malignancy.
Question 2 of 3
2. Question
This is a 32 year old male with a paraspinal mass.
Correct
Answer: D
Histologic Description: This is a highly cellular spindle cell neoplasm, with occasional mitotic figures. The neoplasm permeates bone, which suggests an aggressive process. The neoplasm is diffusely immunoreactive for S100 protein, and other areas demonstrates the hyalinized vessels and lower cellularity typical of schwannoma.
Differential Diagnosis: Rhabdomyosarcoma would label for myogenin and desmin, it would have more eosinophilic cytoplasm. Leiomyosarcoma would demonstrate more eosinophilic cytoplasm and label for desmin. Malignant peripheral nerve sheath tumors are typically not diffusely S100 positive like schwannoma, and demonstrate greater atypia and mitotic activity.
Cellular schwannomas may erode bone and demonstrate mitotic activity, mimicking a malignancy.
Incorrect
Answer: D
Histologic Description: This is a highly cellular spindle cell neoplasm, with occasional mitotic figures. The neoplasm permeates bone, which suggests an aggressive process. The neoplasm is diffusely immunoreactive for S100 protein, and other areas demonstrates the hyalinized vessels and lower cellularity typical of schwannoma.
Differential Diagnosis: Rhabdomyosarcoma would label for myogenin and desmin, it would have more eosinophilic cytoplasm. Leiomyosarcoma would demonstrate more eosinophilic cytoplasm and label for desmin. Malignant peripheral nerve sheath tumors are typically not diffusely S100 positive like schwannoma, and demonstrate greater atypia and mitotic activity.
Cellular schwannomas may erode bone and demonstrate mitotic activity, mimicking a malignancy.
Question 3 of 3
3. Question
This is a 32 year old male with a paraspinal mass
Correct
Answer: D
Histologic Description: This is a highly cellular spindle cell neoplasm, with occasional mitotic figures. The neoplasm permeates bone, which suggests an aggressive process. The neoplasm is diffusely immunoreactive for S100 protein, and other areas demonstrates the hyalinized vessels and lower cellularity typical of schwannoma.
Differential Diagnosis: Rhabdomyosarcoma would label for myogenin and desmin, it would have more eosinophilic cytoplasm. Leiomyosarcoma would demonstrate more eosinophilic cytoplasm and label for desmin. Malignant peripheral nerve sheath tumors are typically not diffusely S100 positive like schwannoma, and demonstrate greater atypia and mitotic activity.
Cellular schwannomas may erode bone and demonstrate mitotic activity, mimicking a malignancy.
Incorrect
Answer: D
Histologic Description: This is a highly cellular spindle cell neoplasm, with occasional mitotic figures. The neoplasm permeates bone, which suggests an aggressive process. The neoplasm is diffusely immunoreactive for S100 protein, and other areas demonstrates the hyalinized vessels and lower cellularity typical of schwannoma.
Differential Diagnosis: Rhabdomyosarcoma would label for myogenin and desmin, it would have more eosinophilic cytoplasm. Leiomyosarcoma would demonstrate more eosinophilic cytoplasm and label for desmin. Malignant peripheral nerve sheath tumors are typically not diffusely S100 positive like schwannoma, and demonstrate greater atypia and mitotic activity.
Cellular schwannomas may erode bone and demonstrate mitotic activity, mimicking a malignancy.
Presented by Dr. Argani and prepared by J. Stephen Nix
This is an 18 year old male with a breast mass.
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This is an 18 year old male with a breast mass.
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Histologic Description: This is an infiltrative ductal process associated with an irregular border and mild stromal desmoplasia. The cells form tubules and cribriform structures and are associated with intracellular and extracellular eosinophilic secretions. The neoplasm demonstrates minimal immunoreactivity for estrogen receptor and progesterone receptor, unlike most low nuclear grade breast cancers. FISH demonstrates rearrangement of the ETV6 gene, supporting the diagnosis of invasive secretory carcinoma.
Differential Diagnosis: Conventional invasive ductal carcinoma would be distinctly unusual in this age group and gender, and does not harbor ETV6 gene rearrangements. Florid papillomatosis of the nipple would have an overall circumscribed appearance, and have bilayered epithelium containing both myoepithelial and ductal cells. Gynecomastia would feature prominent periductal edema and fibrosis, associated with micropapillary duct hyperplasia.
Invasive secretory carcinoma is a special favorable type of breast cancer associated with the ETV6-NTRK3 gene fusion. The latter is also seen in a variety of other neoplasms, including infantile fibrosarcoma, cellular congenital mesoblastic lymphoma, and mammary analog secretory carcinoma of salivary glands. The latter is histologically identical to secretory carcinoma of the breast.
Incorrect
Histologic Description: This is an infiltrative ductal process associated with an irregular border and mild stromal desmoplasia. The cells form tubules and cribriform structures and are associated with intracellular and extracellular eosinophilic secretions. The neoplasm demonstrates minimal immunoreactivity for estrogen receptor and progesterone receptor, unlike most low nuclear grade breast cancers. FISH demonstrates rearrangement of the ETV6 gene, supporting the diagnosis of invasive secretory carcinoma.
Differential Diagnosis: Conventional invasive ductal carcinoma would be distinctly unusual in this age group and gender, and does not harbor ETV6 gene rearrangements. Florid papillomatosis of the nipple would have an overall circumscribed appearance, and have bilayered epithelium containing both myoepithelial and ductal cells. Gynecomastia would feature prominent periductal edema and fibrosis, associated with micropapillary duct hyperplasia.
Invasive secretory carcinoma is a special favorable type of breast cancer associated with the ETV6-NTRK3 gene fusion. The latter is also seen in a variety of other neoplasms, including infantile fibrosarcoma, cellular congenital mesoblastic lymphoma, and mammary analog secretory carcinoma of salivary glands. The latter is histologically identical to secretory carcinoma of the breast.
Presented by Dr. Argani and prepared by J. Stephen Nix
This is a 43 year old female with a renal mass.
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1. Question
This is a 43 year old female with a renal mass.
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Answer: C
Histologic Description: This renal epithelial neoplasm is composed of mainly eosinophilic cells. The cells have variable cytoplasm. Some have voluminous cytoplasm, but other are smaller and focally clustered around hyaline material. The neoplasm demonstrated patchy immunoreactivity for HMB45, diffuse labeling for cathepsin K, and demonstrated a TFEB gene rearrangement by FISH.
Differential Diagnosis: SDHB associated renal cell carcinomas have eosinophilic cytoplasm, but typically demonstrate hyaline cytoplasmic inclusions and vacuolization. Fumarate hydratase deficient renal cell carcinomas typically demonstrate strikingly prominent nucleoli with perinucleolar halos. Oncocytomas typically have a more nested pattern, and lack the nuclear variations seen in the current case.
t(6;11) renal cell carcinomas harbor a MALAT1-TFEB gene fusion. They are included with the Xp11 translocation carcinomas in the family MiT family translocation carcinomas of the kidney.
Reference Argani P. MiT family translocation renal cell carcinomas. Seminars in Diagnostic Pathology 2015; 32:103-113.
Incorrect
Answer: C
Histologic Description: This renal epithelial neoplasm is composed of mainly eosinophilic cells. The cells have variable cytoplasm. Some have voluminous cytoplasm, but other are smaller and focally clustered around hyaline material. The neoplasm demonstrated patchy immunoreactivity for HMB45, diffuse labeling for cathepsin K, and demonstrated a TFEB gene rearrangement by FISH.
Differential Diagnosis: SDHB associated renal cell carcinomas have eosinophilic cytoplasm, but typically demonstrate hyaline cytoplasmic inclusions and vacuolization. Fumarate hydratase deficient renal cell carcinomas typically demonstrate strikingly prominent nucleoli with perinucleolar halos. Oncocytomas typically have a more nested pattern, and lack the nuclear variations seen in the current case.
t(6;11) renal cell carcinomas harbor a MALAT1-TFEB gene fusion. They are included with the Xp11 translocation carcinomas in the family MiT family translocation carcinomas of the kidney.
Reference Argani P. MiT family translocation renal cell carcinomas. Seminars in Diagnostic Pathology 2015; 32:103-113.
Presented by Dr. Argani and prepared by J. Stephen Nix
This is a 2 year old female with a liver mass.
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This is a 2 year old female with a liver mass.
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Answer: A
Histologic Description: This a cystic lesion lined by bland biliary type epithelium. The walls of the cysts are fibrotic. The lesion is well delineated, and the biliary epithelium is indented and distorted by the fibrotic septa. These are the typical features of a mesenchymal hamartoma.
Differential Diagnosis: Embryonal sarcoma of the liver contains anaplastic sarcomatoid cells not seen in the current case. Undifferentiated embryonal sarcoma of the liver may arise in mesenchymal hamartoma, as they share a specific t(11;19) (q13;q13.4) translocation involving the MALAT1 and MHLD1 genes. Hemangiomas would have dilated spaces lined by endothelial cells. Hepatoblastoma would features primitive malignant neoplastic hepatocytes, often associated with extramedullary hematopoiesis.
Mesenchymal hamartoma typically develops in utero and presents before the age of 5 years. On imaging and grossly it presents as a complex cysts. As mentioned before, it may be associated with undifferentiated embryonal sarcoma of the liver.
Incorrect
Answer: A
Histologic Description: This a cystic lesion lined by bland biliary type epithelium. The walls of the cysts are fibrotic. The lesion is well delineated, and the biliary epithelium is indented and distorted by the fibrotic septa. These are the typical features of a mesenchymal hamartoma.
Differential Diagnosis: Embryonal sarcoma of the liver contains anaplastic sarcomatoid cells not seen in the current case. Undifferentiated embryonal sarcoma of the liver may arise in mesenchymal hamartoma, as they share a specific t(11;19) (q13;q13.4) translocation involving the MALAT1 and MHLD1 genes. Hemangiomas would have dilated spaces lined by endothelial cells. Hepatoblastoma would features primitive malignant neoplastic hepatocytes, often associated with extramedullary hematopoiesis.
Mesenchymal hamartoma typically develops in utero and presents before the age of 5 years. On imaging and grossly it presents as a complex cysts. As mentioned before, it may be associated with undifferentiated embryonal sarcoma of the liver.
Presented by Dr. Argani and prepared by J. Stephen Nix
This is a 7 year old female with a thigh mass.
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This is a 7 year old female with a thigh mass.
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Answer: B
Histologic Description: This is a nested neoplasm composed of epithelioid cells with clear cytoplasm. The nuclei are hyperchromatic and have irregular contours, and mitotic figures are relatively easy to identify. The stroma is sclerotic, and there are areas of necrosis. The neoplasm demonstrated diffuse immunoreactivity for S100 protein, and patchy staining for cytokeratins Cam5.2 and AE13. Sequencing revealed and EWSR1-POU5F1 gene fusion, consistent with myoepithelial carcinoma.
Differential Diagnosis: Clear cell hidradenoma would lack the atypia seen in the current case. The lesional cells have clear cytoplasm, and should resemble those of benign urothelium. Epithelioid sarcoma would typically demonstrate a more solid architecture associated with necrosis, and would demonstrate diffuse immunoreactivity for cytokeratin. Clear cell sarcoma of tendon sheath would label for S100, but should also label more strongly for HMB45 and not label for cytokeratins.
The distinction between myoepithelioma and myoepithelial carcinoma in soft tissue is based upon cytologic atypia, not invasion as is used in the salivary gland.
Incorrect
Answer: B
Histologic Description: This is a nested neoplasm composed of epithelioid cells with clear cytoplasm. The nuclei are hyperchromatic and have irregular contours, and mitotic figures are relatively easy to identify. The stroma is sclerotic, and there are areas of necrosis. The neoplasm demonstrated diffuse immunoreactivity for S100 protein, and patchy staining for cytokeratins Cam5.2 and AE13. Sequencing revealed and EWSR1-POU5F1 gene fusion, consistent with myoepithelial carcinoma.
Differential Diagnosis: Clear cell hidradenoma would lack the atypia seen in the current case. The lesional cells have clear cytoplasm, and should resemble those of benign urothelium. Epithelioid sarcoma would typically demonstrate a more solid architecture associated with necrosis, and would demonstrate diffuse immunoreactivity for cytokeratin. Clear cell sarcoma of tendon sheath would label for S100, but should also label more strongly for HMB45 and not label for cytokeratins.
The distinction between myoepithelioma and myoepithelial carcinoma in soft tissue is based upon cytologic atypia, not invasion as is used in the salivary gland.
Presented by Dr. Epstein and prepared by J. Stephen Nix
A 44 year old man was found to have a bladder mass diagnosed in TURB
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A 44 year old man was found to have a bladder mass diagnosed in TURB
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Answer: B
Histology:
The tumor has a variable appearance. Areas of the tumor is composed of clusters of cells with a high nuclear to cytoplasmic ratio giving it the appearance of a “small blue cell tumor”. Other areas consist of markedly pleomorphic tumor giant cells with atypical mitotic figures. A minor part of the tumor shows spindled cells with tapering eosinophilic cytoplasm.
Discussion:
The most common sarcoma in the bladder in an adult is a leiomyosarcoma. Even when very high grade, leiomyosarcomas tend to preserve their architectural pattern of long fascicles of spindle cells cut in both longitudinal and cross-section, which is lacking in the current case. Most tumors in the bladder with a haphazard array of malignant spindle cells are sarcomatoid carcinomas which is synonymous with carcinosarcoma. The diagnosis is established by either demonstrating keratin expression or by finding an associated either in-situ or infiltrating carcinoma component. Although areas of this case could resemble small cell carcinoma, the very pleomorphic areas and the spindle cell features are against this diagnosis. The clue to the correct diagnosis of rhabdomyosarcoma is the presence of some cells with long eosinophilic cytoplasmic tails and to recognize. In addition, it must be recognized that although bladder rhabdomyosarcomas are typically seen in children they can also occur in adults. A pitfall is that bladder rhabdomyosarcomas can express CD56 and synaptophysin mimicking small cell carcinoma. One must keep rhabdomyosarcoma in the differential diagnosis and establish the diagnosis with immunohistochemistry for myogenin.
Incorrect
Answer: B
Histology:
The tumor has a variable appearance. Areas of the tumor is composed of clusters of cells with a high nuclear to cytoplasmic ratio giving it the appearance of a “small blue cell tumor”. Other areas consist of markedly pleomorphic tumor giant cells with atypical mitotic figures. A minor part of the tumor shows spindled cells with tapering eosinophilic cytoplasm.
Discussion:
The most common sarcoma in the bladder in an adult is a leiomyosarcoma. Even when very high grade, leiomyosarcomas tend to preserve their architectural pattern of long fascicles of spindle cells cut in both longitudinal and cross-section, which is lacking in the current case. Most tumors in the bladder with a haphazard array of malignant spindle cells are sarcomatoid carcinomas which is synonymous with carcinosarcoma. The diagnosis is established by either demonstrating keratin expression or by finding an associated either in-situ or infiltrating carcinoma component. Although areas of this case could resemble small cell carcinoma, the very pleomorphic areas and the spindle cell features are against this diagnosis. The clue to the correct diagnosis of rhabdomyosarcoma is the presence of some cells with long eosinophilic cytoplasmic tails and to recognize. In addition, it must be recognized that although bladder rhabdomyosarcomas are typically seen in children they can also occur in adults. A pitfall is that bladder rhabdomyosarcomas can express CD56 and synaptophysin mimicking small cell carcinoma. One must keep rhabdomyosarcoma in the differential diagnosis and establish the diagnosis with immunohistochemistry for myogenin.
Presented by Dr. Epstein and prepared by J. Stephen Nix
A 56 year old female underwent a partial nephrectomy for a solid renal mass
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A 56 year old female underwent a partial nephrectomy for a solid renal mass
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Answer: B
Histology:
The tumor consists of a sheet of eosinophilic cells. The cells have low grade cytology with uniform round nuclei and indistinct nucleoli. Many of the cells have a distinct cytoplasmic inclusion which is round and pale eosinophilic.
Discussion:
Oncocytomas architecturally do not form sheets of cells. Rather, they are composed of nests of cells that are typically present in a fibromyxoid matrix. At low power, occasionally oncocytomas may appear diffuse but on closer inspection have back-to-back nests of cells. Chromophobe RCC does tend to form sheets of cells with tubular formation along with nests and typically lacking the fibromyxoid background. Characteristically, some of the cells in chromophobe RCC will have irregular crinkly, notched, or cleaved nuclei, whereas in oncocytoma the nuclei are round. In the current case, the characteristic finding are the pale cytoplasmic inclusions which are typical of SDHB deficient RCC. The diagnosis was confirmed with immunohistochemistry showing a loss of SDHB expression in the tumor. The importance of establishing this diagnosis is that a minority of SDHB deficient RCCs are associated with the autosomal dominant hereditary paraganglioma syndrome which has in addition to multiple paragangliomas is also associated with GISTs typically in the stomach with a predilection for children. Although these tumors classically are low grade and have the inclusions, there is a spectrum in their morphology such that SDHB immunohistochemistry is often performed in unclassified low grade eosinophilic RCCs.
Incorrect
Answer: B
Histology:
The tumor consists of a sheet of eosinophilic cells. The cells have low grade cytology with uniform round nuclei and indistinct nucleoli. Many of the cells have a distinct cytoplasmic inclusion which is round and pale eosinophilic.
Discussion:
Oncocytomas architecturally do not form sheets of cells. Rather, they are composed of nests of cells that are typically present in a fibromyxoid matrix. At low power, occasionally oncocytomas may appear diffuse but on closer inspection have back-to-back nests of cells. Chromophobe RCC does tend to form sheets of cells with tubular formation along with nests and typically lacking the fibromyxoid background. Characteristically, some of the cells in chromophobe RCC will have irregular crinkly, notched, or cleaved nuclei, whereas in oncocytoma the nuclei are round. In the current case, the characteristic finding are the pale cytoplasmic inclusions which are typical of SDHB deficient RCC. The diagnosis was confirmed with immunohistochemistry showing a loss of SDHB expression in the tumor. The importance of establishing this diagnosis is that a minority of SDHB deficient RCCs are associated with the autosomal dominant hereditary paraganglioma syndrome which has in addition to multiple paragangliomas is also associated with GISTs typically in the stomach with a predilection for children. Although these tumors classically are low grade and have the inclusions, there is a spectrum in their morphology such that SDHB immunohistochemistry is often performed in unclassified low grade eosinophilic RCCs.
Presented by Dr. Epstein and prepared by J. Stephen Nix
A 44 year old male underwent an orchiectomy for a 5 cm testicular mass
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A 44 year old male underwent an orchiectomy for a 5 cm testicular mass
Correct
Answer: D
Histology:
The mass is composed of cords and nests of cells with abundant eosinophilic cytoplasm. Nuclei are enlarged, hyperchromatic, with variable shapes and prominent nucleoli. Mitotic figures are easily identifiable and necrosis is present. Scattered throughout out the tumor are small foci of irregular calcification.
Discussion:
The findings in this case are classic for a large cell calcifying Sertoli cell tumor. Although the tumor is composed of cells that resemble Leydig cells, it is classified as a Sertoli cell tumor since these lesions can express keratin and can have an intratubular component – as Leydig cells reside between seminiferous tubules a Leydig cell tumor would not be expected to be intratubular. The finding of irregular deposits of calcification is unique within the testis for large cell calcifying Sertoli cell tumor. The same criteria for malignancy in these tumors are those for other sex-cord stromal tumors based on size, mitotic figures, necrosis, atypia, and widespread infiltration. Approximately 10% of large cell calcifying Sertoli cell tumors are malignant. Although the majority of large cell calcifying Sertoli cell tumors are sporadic some are associated with the Carney complex which is an autosomal dominant syndrome which has in addition pituitary adenomas, myxomas, pigmented nodular adrenocortical hyperplasia, and skin pigmentation. The best immunohistochemical marker for sex cord stromal tumors in general is SF-1 and if not available inhibin.
Incorrect
Answer: D
Histology:
The mass is composed of cords and nests of cells with abundant eosinophilic cytoplasm. Nuclei are enlarged, hyperchromatic, with variable shapes and prominent nucleoli. Mitotic figures are easily identifiable and necrosis is present. Scattered throughout out the tumor are small foci of irregular calcification.
Discussion:
The findings in this case are classic for a large cell calcifying Sertoli cell tumor. Although the tumor is composed of cells that resemble Leydig cells, it is classified as a Sertoli cell tumor since these lesions can express keratin and can have an intratubular component – as Leydig cells reside between seminiferous tubules a Leydig cell tumor would not be expected to be intratubular. The finding of irregular deposits of calcification is unique within the testis for large cell calcifying Sertoli cell tumor. The same criteria for malignancy in these tumors are those for other sex-cord stromal tumors based on size, mitotic figures, necrosis, atypia, and widespread infiltration. Approximately 10% of large cell calcifying Sertoli cell tumors are malignant. Although the majority of large cell calcifying Sertoli cell tumors are sporadic some are associated with the Carney complex which is an autosomal dominant syndrome which has in addition pituitary adenomas, myxomas, pigmented nodular adrenocortical hyperplasia, and skin pigmentation. The best immunohistochemical marker for sex cord stromal tumors in general is SF-1 and if not available inhibin.
Presented by Dr. Matoso and prepared by J. Stephen Nix
60 yo male with kidney tumor
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60 yo male with kidney tumor
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Correct C
Histology:
Well circumscribed mass composed of anastomosing sinusoidal capillary sized vessels with hobnail endothelial cells. There is focal fibrin deposition and scattered megakaryocytes as evidence of extramedullary hematopoiesis. There is no significant atypia, necrosis or mitotic activity.
Discussion:
Anastomosing hemangiomas are uncommon but well documented in the genitourinary tract. When present in the kidney, they are more commonly involving the cortical area and are solitary. The differential diagnosis includes clear cell renal cell carcinoma with prominent vasculature, hemangioblastoma and angiosarcoma. There is no epithelial component in this case excluding RCC. This case also lacks the large vacuolated stromal cells characteristic of hemangioblastomas. Angiosarcoma is excluded given the lack of significant atypia, necrosis and mitotic activity. Anastomosing hemangiomas are benign and the cured by excision.
Well circumscribed mass composed of anastomosing sinusoidal capillary sized vessels with hobnail endothelial cells. There is focal fibrin deposition and scattered megakaryocytes as evidence of extramedullary hematopoiesis. There is no significant atypia, necrosis or mitotic activity.
Discussion:
Anastomosing hemangiomas are uncommon but well documented in the genitourinary tract. When present in the kidney, they are more commonly involving the cortical area and are solitary. The differential diagnosis includes clear cell renal cell carcinoma with prominent vasculature, hemangioblastoma and angiosarcoma. There is no epithelial component in this case excluding RCC. This case also lacks the large vacuolated stromal cells characteristic of hemangioblastomas. Angiosarcoma is excluded given the lack of significant atypia, necrosis and mitotic activity. Anastomosing hemangiomas are benign and the cured by excision.
Presented by Dr. Matoso and prepared by J. Stephen Nix.
32 yo male with testicular tumor
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32 yo male with testicular tumor.
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Correct D
Histology:
The tumor is composed of a mixed population of small blue cells with marked nuclear atypia and fibrillary background and an epithelial component forming glandular spaces. There is necrosis and nuclear pseudopallisading in areas within the small blue cell component.
Discussion:
Neoplastic neuroglial differentiation outside of the central nervous system is rare and has been reported mainly in association with teratomas, the great majority of them ovarian or gynecologic in origin. In men, testicular tumors with neuroglial neoplasms are extremely rare. neuroglial differentiation and neoplasms are rare in testicular germ cell tumors and are most commonly associated with teratomas; they can be seen in primary and metastatic sites. They exhibit the full range of neuroglial differentiation including developing CNS to gliomas/glioneuronal tumors WHO grades I-IV. None of the cases showed results consistent with ATRX, IDH or BRAF alterations, suggesting they have different oncogenic mechanisms than their CNS counterparts. This case was diagnosed as glioblastoma (WHO grade IV) arising in a teratoma based on the presence of marked nuclear pleomorphism, foci of necrosis, and positive immunostaining for GFAP and OLIG2.
References:
Matoso A, Idrees MT, Rodriguez FJ, Ibrahim J, Perrino CM, Ulbright TM, Epstein JI. Neuroglial Differentiation and Neoplasms in Testicular Germ Cell Tumors Lack Immunohistochemical Evidence of Alterations Characteristic of Their CNS Counterparts: A Study of 13 Cases. Am J Surg Pathol. 2018.
Sangruchi T, Sobel RA. Microglial and neural differentiation in human teratomas. Acta Neuropathol. 1989;78:258-263.
Incorrect
Correct D
Histology:
The tumor is composed of a mixed population of small blue cells with marked nuclear atypia and fibrillary background and an epithelial component forming glandular spaces. There is necrosis and nuclear pseudopallisading in areas within the small blue cell component.
Discussion:
Neoplastic neuroglial differentiation outside of the central nervous system is rare and has been reported mainly in association with teratomas, the great majority of them ovarian or gynecologic in origin. In men, testicular tumors with neuroglial neoplasms are extremely rare. neuroglial differentiation and neoplasms are rare in testicular germ cell tumors and are most commonly associated with teratomas; they can be seen in primary and metastatic sites. They exhibit the full range of neuroglial differentiation including developing CNS to gliomas/glioneuronal tumors WHO grades I-IV. None of the cases showed results consistent with ATRX, IDH or BRAF alterations, suggesting they have different oncogenic mechanisms than their CNS counterparts. This case was diagnosed as glioblastoma (WHO grade IV) arising in a teratoma based on the presence of marked nuclear pleomorphism, foci of necrosis, and positive immunostaining for GFAP and OLIG2.
References:
Matoso A, Idrees MT, Rodriguez FJ, Ibrahim J, Perrino CM, Ulbright TM, Epstein JI. Neuroglial Differentiation and Neoplasms in Testicular Germ Cell Tumors Lack Immunohistochemical Evidence of Alterations Characteristic of Their CNS Counterparts: A Study of 13 Cases. Am J Surg Pathol. 2018.
Sangruchi T, Sobel RA. Microglial and neural differentiation in human teratomas. Acta Neuropathol. 1989;78:258-263.
Presented by Dr. Matoso and prepared by J. Stephen Nix.
54 yo male with testicular tumor
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54 yo male with testicular tumor.
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Correct: B
Histology: Non-encapsulated well-circumscribed tumor composed of tubules and nests of tumor cells with small or inconspicuous nucleoli and clear cytoplasm in a background of hyalinized stroma.
Discussion: At low power, the clear cytoplasm of the tumor can mimic a seminoma. Unlike seminoma, the nuclei have small nucleoli and the tumor lacks the lymphocytic infiltration of seminoma. Also important is the presence of sclerosed stroma in between tumor cells. Sertoli cell tumors are infrequent and constitute less than 1% of all testicular tumors. The most frequent clinical presentation is a painless testicular mass. Occasionally, patients will present with gynecomastia. The majority are sporadic but some are associated with androgen insensitivity syndrome, Carney syndrome or Peutz-Jeghers syndrome. Cord-like arrangements of the cells in scanty pauci-cellular stroma could mimic a neuroendocrine tumor, especially because a subset of Sertoli cell tumors are positive for neuroendocrine markers. Inhibin is positive but less frequently than in Leydig cell tumors. Other positive markers include EMA and SF1. Germ cell markers including PLAP, OCT3/4 and CD30 are negative. Size over 5 cm, cellular atypia, increased mitotic activity, necrosis, infiltrative pattern, extratesticular extension and vascular invasion are associated with malignancy.
Histology: Non-encapsulated well-circumscribed tumor composed of tubules and nests of tumor cells with small or inconspicuous nucleoli and clear cytoplasm in a background of hyalinized stroma.
Discussion: At low power, the clear cytoplasm of the tumor can mimic a seminoma. Unlike seminoma, the nuclei have small nucleoli and the tumor lacks the lymphocytic infiltration of seminoma. Also important is the presence of sclerosed stroma in between tumor cells. Sertoli cell tumors are infrequent and constitute less than 1% of all testicular tumors. The most frequent clinical presentation is a painless testicular mass. Occasionally, patients will present with gynecomastia. The majority are sporadic but some are associated with androgen insensitivity syndrome, Carney syndrome or Peutz-Jeghers syndrome. Cord-like arrangements of the cells in scanty pauci-cellular stroma could mimic a neuroendocrine tumor, especially because a subset of Sertoli cell tumors are positive for neuroendocrine markers. Inhibin is positive but less frequently than in Leydig cell tumors. Other positive markers include EMA and SF1. Germ cell markers including PLAP, OCT3/4 and CD30 are negative. Size over 5 cm, cellular atypia, increased mitotic activity, necrosis, infiltrative pattern, extratesticular extension and vascular invasion are associated with malignancy.
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