Presented by Dr. Pedram Argani and prepared by Dr. Yembur Ahmad
This case talks about an adult patient with a renal mass.
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This is an adult patient with a 6 cm renal mass. What is the diagnosis?
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Answer: A
Histologic Description: This is a cellular neoplasm composed of spindle and epithelioid cells. There are strands of collagen throughout the lesion that have a ropy quality. The neoplasm was diffusely immunoreactive for CD34 and demonstrated STAT6 nuclear labeling, supporting the diagnosis of cellular solitary fibrous tumor.
Differential Diagnosis: Synovial sarcoma will not label for CD34 and STAT6, and typically demonstrates focal cytokeratin immunoreactivity. Like the current lesion, clear cell sarcoma of the kidney could label for BCOR, but would have much more open chromatin and would be extremely uncommon in a patient of this age. Sarcomatoid renal cell carcinoma would be defined by seeing a well-defined epithelioid renal cell carcinoma component in addition to malignant spindle cells.
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Answer: A
Histologic Description: This is a cellular neoplasm composed of spindle and epithelioid cells. There are strands of collagen throughout the lesion that have a ropy quality. The neoplasm was diffusely immunoreactive for CD34 and demonstrated STAT6 nuclear labeling, supporting the diagnosis of cellular solitary fibrous tumor.
Differential Diagnosis: Synovial sarcoma will not label for CD34 and STAT6, and typically demonstrates focal cytokeratin immunoreactivity. Like the current lesion, clear cell sarcoma of the kidney could label for BCOR, but would have much more open chromatin and would be extremely uncommon in a patient of this age. Sarcomatoid renal cell carcinoma would be defined by seeing a well-defined epithelioid renal cell carcinoma component in addition to malignant spindle cells.
Presented by Dr. Pedram Argani and prepared by Dr. Yembur Ahmad
This case talks about bilateral renal tumors in a pediatric patient.
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A young child presents with bilateral renal tumors after therapy. The tumor illustrated is the largest one. What is the diagnosis?
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Answer: C
Histologic Description: This is a post-therapy specimen, so there are large areas of regression consisting of macrophages and scarring. There are also areas of differentiated epithelium at the periphery of the tumor. Other areas show the usual proliferating blastemal elements that are non-anaplastic. However, there is well-defined nodule of blastemal cells with markedly enlarged nuclei showing hyperchromasia, along with atypical mitotic figures. This focus was well delineated and confined to the kidney, which justifies the diagnosis of focal anaplasia.
Differential Diagnosis: The presence of enlarged hyperchromatic nuclei demonstrating atypical mitotic figures supports the diagnosis of anaplasia (unfavorable histology). Focal anaplasia must be well delineated and confined to the kidney, as seen in the current case. If not, anaplasia is considered diffuse by default. Desmoplastic small round cell tumor is immunoreactive for WT1, desmin and keratin like the current Wilms tumor; however, it typically demonstrates desmoplastic stroma and nuclei that have coarser chromatin than the delicate chromatin of a blastemal Wilms tumor nucleus.
Incorrect
Answer: C
Histologic Description: This is a post-therapy specimen, so there are large areas of regression consisting of macrophages and scarring. There are also areas of differentiated epithelium at the periphery of the tumor. Other areas show the usual proliferating blastemal elements that are non-anaplastic. However, there is well-defined nodule of blastemal cells with markedly enlarged nuclei showing hyperchromasia, along with atypical mitotic figures. This focus was well delineated and confined to the kidney, which justifies the diagnosis of focal anaplasia.
Differential Diagnosis: The presence of enlarged hyperchromatic nuclei demonstrating atypical mitotic figures supports the diagnosis of anaplasia (unfavorable histology). Focal anaplasia must be well delineated and confined to the kidney, as seen in the current case. If not, anaplasia is considered diffuse by default. Desmoplastic small round cell tumor is immunoreactive for WT1, desmin and keratin like the current Wilms tumor; however, it typically demonstrates desmoplastic stroma and nuclei that have coarser chromatin than the delicate chromatin of a blastemal Wilms tumor nucleus.
Presented by Dr. Pedram Argani and prepared by Dr. Yembur Ahmad
This case is about a young adult patient with a renal tumor.
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A young adult patient presents with a renal tumor showing sinus vascular invasion. What is the diagnosis?
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Answer: B
Histologic Description: The neoplasm is separated by fibrous septa which have a hyalinized appearance. The neoplastic cells have well defined cell borders and irregular nuclei with perinuclear halos. There is abundant psammomatous calcification present. The latter suggests the possibility of Xp11 translocation RCC, but can be seen in chromophobe RCC. The neoplasm demonstrated diffuse membranous labeling for CD117, supporting the diagnosis of chromophobe RCC.
Differential Diagnosis: Xp11 translocation RCC does not usually show diffuse membranous labeling for CD117, and would label for TFE3. Clear cell RCC would have more vascularized septa, and lack the prominent perinuclear halos and nuclear irregularities of this case. t(6;11) renal cell carcinomas typically have a biphasic large cell /small cell morphology, and label for melanocytic markers like Melan A.
Incorrect
Answer: B
Histologic Description: The neoplasm is separated by fibrous septa which have a hyalinized appearance. The neoplastic cells have well defined cell borders and irregular nuclei with perinuclear halos. There is abundant psammomatous calcification present. The latter suggests the possibility of Xp11 translocation RCC, but can be seen in chromophobe RCC. The neoplasm demonstrated diffuse membranous labeling for CD117, supporting the diagnosis of chromophobe RCC.
Differential Diagnosis: Xp11 translocation RCC does not usually show diffuse membranous labeling for CD117, and would label for TFE3. Clear cell RCC would have more vascularized septa, and lack the prominent perinuclear halos and nuclear irregularities of this case. t(6;11) renal cell carcinomas typically have a biphasic large cell /small cell morphology, and label for melanocytic markers like Melan A.
Presented by Dr. Andres Matoso and prepared by Dr. Yembur Ahmad
A 72 year old male with an abnormal prostate exam but normal PSA.
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A 72 year old male presents with an abnormal prostate exam, but normal PSA. What is the diagnosis?
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Correct Answer: B
Histology: The lesion consists of glands with solid nests composed of cells with basophilic nuclei with scant cytoplasm. Some of the nests have a small glandular lumina. There is no necrosis or reaction of the stroma.
Discussion: Basal cell hyperplasia consists of glands with basophilic nuclei and scant cytoplasm and increased number of the basal layer, from a double-layer up to solid nest. It can range from small focal areas to florid. Glands can appear to form cribriform/pseudocribriform structures, but are actually small round hyperplastic glands crowded together. Well-formed lamellar intraluminal calcifications can be observed, which is extremely rare in carcinoma. Intracytoplasmic eosinophilic globules can also be present. The most frequent location is in the transition zone and therefore are more commonly seen in transurethral resections. In needle biopsies, the glands may appear to have an infiltrative growth pattern. Basal cell hyperplasia may have prominent nucleoli, further mimicking cancer. If cancer is suspected, immunohistochemistry is helpful in the differential diagnosis with adenocarcinoma because BCH is positive for basal cell markers, and negative for racemase. The differential diagnosis of BCH should also include basal cell carcinoma. The features that are seen in carcinoma but not in hyperplasia include large nests with necrosis, adenoid cystic pattern, variable sizes and shapes of the nests with infiltrating borders, extraprostatic extension or bladder neck invasion, and desmoplastic stromal reaction. Occasionally, basal cell hyperplasia can involve bladder neck muscle or present between normal prostatic glands, therefore these findings should not be considered in isolation, sufficient to make a diagnosis of carcinoma. By immunohistochemistry, diffuse staining for Bcl-2 and elevated Ki67 correlate have been reported to be associated with carcinoma and not hyperplasia.
References:
Am J Surg Pathol. 2002;26(2):237-243.
Am J Surg Pathol. 1992;16(12):1205-1214.
Hum Pathol. 2005;36(5):480-485.
Incorrect
Correct Answer: B
Histology: The lesion consists of glands with solid nests composed of cells with basophilic nuclei with scant cytoplasm. Some of the nests have a small glandular lumina. There is no necrosis or reaction of the stroma.
Discussion: Basal cell hyperplasia consists of glands with basophilic nuclei and scant cytoplasm and increased number of the basal layer, from a double-layer up to solid nest. It can range from small focal areas to florid. Glands can appear to form cribriform/pseudocribriform structures, but are actually small round hyperplastic glands crowded together. Well-formed lamellar intraluminal calcifications can be observed, which is extremely rare in carcinoma. Intracytoplasmic eosinophilic globules can also be present. The most frequent location is in the transition zone and therefore are more commonly seen in transurethral resections. In needle biopsies, the glands may appear to have an infiltrative growth pattern. Basal cell hyperplasia may have prominent nucleoli, further mimicking cancer. If cancer is suspected, immunohistochemistry is helpful in the differential diagnosis with adenocarcinoma because BCH is positive for basal cell markers, and negative for racemase. The differential diagnosis of BCH should also include basal cell carcinoma. The features that are seen in carcinoma but not in hyperplasia include large nests with necrosis, adenoid cystic pattern, variable sizes and shapes of the nests with infiltrating borders, extraprostatic extension or bladder neck invasion, and desmoplastic stromal reaction. Occasionally, basal cell hyperplasia can involve bladder neck muscle or present between normal prostatic glands, therefore these findings should not be considered in isolation, sufficient to make a diagnosis of carcinoma. By immunohistochemistry, diffuse staining for Bcl-2 and elevated Ki67 correlate have been reported to be associated with carcinoma and not hyperplasia.
References:
Am J Surg Pathol. 2002;26(2):237-243.
Am J Surg Pathol. 1992;16(12):1205-1214.
Hum Pathol. 2005;36(5):480-485.
Presented by Dr. Andres Matoso and prepared by Dr. Yembur Ahmad
A 64 year old male with a prostate mass.
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A 64 year old male presents with a prostate mass. What is the diagnosis?
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Correct Answer: C
Histology: This prostate tumor shows a proliferation of spindle stroma cells with small nuclei and variable amount of eosinophilic cytoplasm arranged in a whorled of haphazard pattern. The tumor is characterized by having thick walled vessels.
Discussion: One of the most common stromal proliferations identified in prostate needle biopsies are stromal nodules of benign prostatic hyperplasia (BPH). BPH is common among middle age and elder men and it affects up to 80% of men. BPH refers to the proliferation and enlargement of both the stromal and the glandular components but there is evidence that suggests that it is the stroma the one that leads to more pronounced enlargement and obstructive symptoms. By immunohistochemistry, the spindle cells are most commonly positive for vimentin and some cases with more prominent fibromuscular differentiation show positive staining for smooth muscle actin and desmin. The immunohistochemistry profile is not specific and of limited utility in the differential diagnosis. The main differential diagnoses include stromal tumor of uncertain malignant potential (STUMP) which, in contrast to stromal nodule of BPH, has more pronounced nuclear pleomorphism, often infiltrates around glands, and lacks the characteristic thick-walled blood vessels.
Histology: This prostate tumor shows a proliferation of spindle stroma cells with small nuclei and variable amount of eosinophilic cytoplasm arranged in a whorled of haphazard pattern. The tumor is characterized by having thick walled vessels.
Discussion: One of the most common stromal proliferations identified in prostate needle biopsies are stromal nodules of benign prostatic hyperplasia (BPH). BPH is common among middle age and elder men and it affects up to 80% of men. BPH refers to the proliferation and enlargement of both the stromal and the glandular components but there is evidence that suggests that it is the stroma the one that leads to more pronounced enlargement and obstructive symptoms. By immunohistochemistry, the spindle cells are most commonly positive for vimentin and some cases with more prominent fibromuscular differentiation show positive staining for smooth muscle actin and desmin. The immunohistochemistry profile is not specific and of limited utility in the differential diagnosis. The main differential diagnoses include stromal tumor of uncertain malignant potential (STUMP) which, in contrast to stromal nodule of BPH, has more pronounced nuclear pleomorphism, often infiltrates around glands, and lacks the characteristic thick-walled blood vessels.
Presented by Dr. Andres Matoso and prepared by Dr. Yembur Ahmad
75 year old male with an adrenal mass.
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A 75 year old male presents with an adrenal mass. What is your diagnosis?
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Correct answer: A
Histology: This tumor is composed of nests and cords of cells with eosinophilic and pale cytoplasm and marked degenerative nuclear atypia. There is extensive hemorrhage and thrombus formation that makes for the majority of the mass.
Discussion: This is an example of enlarged adrenal glands where the underlying diagnosis can be masked by a diffusely hemorrhagic process. The most common underlying lesion associated with diffuse hemorrhage are adrenocortical adenomas and benign non-neoplastic adrenal. Carcinomas and other rarer lesions can also show similar findings. Although there is a correlation between adrenocortical malignancy and size, hemorrhage into nonmalignant adrenal glands can result in markedly enlarged adrenals. Around the hemorrhage there is a proliferation of cortical cells with degenerative atypia, so called “endocrine atypia” which is a finding not associated with malignancy.
Histology: This tumor is composed of nests and cords of cells with eosinophilic and pale cytoplasm and marked degenerative nuclear atypia. There is extensive hemorrhage and thrombus formation that makes for the majority of the mass.
Discussion: This is an example of enlarged adrenal glands where the underlying diagnosis can be masked by a diffusely hemorrhagic process. The most common underlying lesion associated with diffuse hemorrhage are adrenocortical adenomas and benign non-neoplastic adrenal. Carcinomas and other rarer lesions can also show similar findings. Although there is a correlation between adrenocortical malignancy and size, hemorrhage into nonmalignant adrenal glands can result in markedly enlarged adrenals. Around the hemorrhage there is a proliferation of cortical cells with degenerative atypia, so called “endocrine atypia” which is a finding not associated with malignancy.
Presented by Dr. Andres Matoso and prepared by Dr. Yembur Ahmad
72 year old male with abnormal prostate exam but normal PSA
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A 72 year old male presents with an abnormal prostate exam but normal PSA. What is the diagnosis?
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Correct Answer: C
Histology: The tumor is composed by atypical cells with hyperchromatic nuclei with inconspicuous nucleoli and scant cytoplasm. There is nuclear molding and frequent mitoses and apoptotic cells.
Discussion: Small cell carcinoma is a high-grade NE tumor with morphologic characteristics of small cell carcinoma originated in any other organs. Similar to carcinoid tumors, by definition, small cell carcinoma should be negative for prostate markers. While primary prostatic small cell carcinoma is rare, it is more frequent than carcinoid tumors or large cell neuroendocrine carcinoma. Approximately half of the cases are pure while the other half are mixed small cell carcinoma and adenocarcinoma.1 Because small cell carcinoma does not produce PSA, patients with pure small cell carcinoma have serum PSA levels that are within normal limits or mildly elevated.1 The main differential diagnosis is with adenocarcinoma Gleason score 5+5=10 (Grade Group 5). By definition, small cell carcinoma should lack markers of prostatic differentiation, therefore, immunohistochemistry for prostate specific markers including PSA, NKX3.1 or prostein are negative. Some cases will show a transition from high grade adenocarcinoma into small cell carcinoma where the prostate markers are retained in the area of adenocarcinoma and lost in the small cell carcinoma component. Additionally, the small cell carcinoma component is typically positive for at least one NE marker (synaptophysin, chromogranin, or CD56), and FOXA2. 1-3 In addition to NE markers, the majority (>50%) of small cell carcinoma of the prostate are positive for TTF-1, which makes the differential of metastatic small cell carcinoma not possible to determine the site of origin based on the morphologic and immunohistochemistry findings alone.4,5 A subset of small cell carcinoma that arose from prostate adenocarcinoma will harbor the characteristic gene fusion involving ERG, which can be detected by in-situ hybridization. Immunohistochemistry for ERG is usually negative even in cases of small cell carcinoma that harbor the gene rearrangement.6 Loss of expression of RB has been shown to be characteristic of small cell carcinoma and not conventional adenocarcinoma, but its use alone is not recommended because of high number of false negatives.7 Other less common differential diagnoses that should be considered in cases all cytokeratin stains are negative include lymphoma and poorly differentiated sarcomas.
In cases that are mixed adenocarcinoma and small cell carcinoma of the prostate the small cell carcinoma component should not be included in the grade. Additionally, the adenocarcinoma component should only be graded if it does not show prominent treatment affect.
Small cell carcinoma of the prostate has a poor prognosis with one study showing a median survival rate from the time of diagnosis of 17 months.8
References
1. Wang W, Epstein JI. Small cell carcinoma of the prostate. A morphologic and immunohistochemical study of 95 cases. Am J Surg Pathol. 2008;32(1):65-71.
2. Yao JL, Madeb R, Bourne P, et al. Small cell carcinoma of the prostate: an immunohistochemical study. Am J Surg Pathol. 2006;30(6):705-712.
3. Park JW, Lee JK, Witte ON, Huang J. FOXA2 is a sensitive and specific marker for small cell neuroendocrine carcinoma of the prostate. Mod Pathol. 2017;30(9):1262-1272.
4. Agoff SN, Lamps LW, Philip AT, et al. Thyroid transcription factor-1 is expressed in extrapulmonary small cell carcinomas but not in other extrapulmonary neuroendocrine tumors. Mod Pathol. 2000;13(3):238-242.
5. Ordonez NG. Value of thyroid transcription factor-1 immunostaining in distinguishing small cell lung carcinomas from other small cell carcinomas. Am J Surg Pathol. 2000;24(9):1217-1223.
6. Lotan TL, Gupta NS, Wang W, et al. ERG gene rearrangements are common in prostatic small cell carcinomas. Mod Pathol. 2011;24(6):820-828.
7. Tan HL, Sood A, Rahimi HA, et al. Rb loss is characteristic of prostatic small cell neuroendocrine carcinoma. Clin Cancer Res. 2014;20(4):890-903.
8. Deorah S, Rao MB, Raman R, Gaitonde K, Donovan JF. Survival of patients with small cell carcinoma of the prostate during 1973-2003: a population-based study. BJU Int. 2012;109(6):824-830.
Incorrect
Correct Answer: C
Histology: The tumor is composed by atypical cells with hyperchromatic nuclei with inconspicuous nucleoli and scant cytoplasm. There is nuclear molding and frequent mitoses and apoptotic cells.
Discussion: Small cell carcinoma is a high-grade NE tumor with morphologic characteristics of small cell carcinoma originated in any other organs. Similar to carcinoid tumors, by definition, small cell carcinoma should be negative for prostate markers. While primary prostatic small cell carcinoma is rare, it is more frequent than carcinoid tumors or large cell neuroendocrine carcinoma. Approximately half of the cases are pure while the other half are mixed small cell carcinoma and adenocarcinoma.1 Because small cell carcinoma does not produce PSA, patients with pure small cell carcinoma have serum PSA levels that are within normal limits or mildly elevated.1 The main differential diagnosis is with adenocarcinoma Gleason score 5+5=10 (Grade Group 5). By definition, small cell carcinoma should lack markers of prostatic differentiation, therefore, immunohistochemistry for prostate specific markers including PSA, NKX3.1 or prostein are negative. Some cases will show a transition from high grade adenocarcinoma into small cell carcinoma where the prostate markers are retained in the area of adenocarcinoma and lost in the small cell carcinoma component. Additionally, the small cell carcinoma component is typically positive for at least one NE marker (synaptophysin, chromogranin, or CD56), and FOXA2. 1-3 In addition to NE markers, the majority (>50%) of small cell carcinoma of the prostate are positive for TTF-1, which makes the differential of metastatic small cell carcinoma not possible to determine the site of origin based on the morphologic and immunohistochemistry findings alone.4,5 A subset of small cell carcinoma that arose from prostate adenocarcinoma will harbor the characteristic gene fusion involving ERG, which can be detected by in-situ hybridization. Immunohistochemistry for ERG is usually negative even in cases of small cell carcinoma that harbor the gene rearrangement.6 Loss of expression of RB has been shown to be characteristic of small cell carcinoma and not conventional adenocarcinoma, but its use alone is not recommended because of high number of false negatives.7 Other less common differential diagnoses that should be considered in cases all cytokeratin stains are negative include lymphoma and poorly differentiated sarcomas.
In cases that are mixed adenocarcinoma and small cell carcinoma of the prostate the small cell carcinoma component should not be included in the grade. Additionally, the adenocarcinoma component should only be graded if it does not show prominent treatment affect.
Small cell carcinoma of the prostate has a poor prognosis with one study showing a median survival rate from the time of diagnosis of 17 months.8
References
1. Wang W, Epstein JI. Small cell carcinoma of the prostate. A morphologic and immunohistochemical study of 95 cases. Am J Surg Pathol. 2008;32(1):65-71.
2. Yao JL, Madeb R, Bourne P, et al. Small cell carcinoma of the prostate: an immunohistochemical study. Am J Surg Pathol. 2006;30(6):705-712.
3. Park JW, Lee JK, Witte ON, Huang J. FOXA2 is a sensitive and specific marker for small cell neuroendocrine carcinoma of the prostate. Mod Pathol. 2017;30(9):1262-1272.
4. Agoff SN, Lamps LW, Philip AT, et al. Thyroid transcription factor-1 is expressed in extrapulmonary small cell carcinomas but not in other extrapulmonary neuroendocrine tumors. Mod Pathol. 2000;13(3):238-242.
5. Ordonez NG. Value of thyroid transcription factor-1 immunostaining in distinguishing small cell lung carcinomas from other small cell carcinomas. Am J Surg Pathol. 2000;24(9):1217-1223.
6. Lotan TL, Gupta NS, Wang W, et al. ERG gene rearrangements are common in prostatic small cell carcinomas. Mod Pathol. 2011;24(6):820-828.
7. Tan HL, Sood A, Rahimi HA, et al. Rb loss is characteristic of prostatic small cell neuroendocrine carcinoma. Clin Cancer Res. 2014;20(4):890-903.
8. Deorah S, Rao MB, Raman R, Gaitonde K, Donovan JF. Survival of patients with small cell carcinoma of the prostate during 1973-2003: a population-based study. BJU Int. 2012;109(6):824-830.
Presented by Dr. Andres Matoso and prepared by Dr. Yembur Ahmad
1 day old female with alveolar mass
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A 1 day old baby girl presents with an alveolar mass. What is the diagnosis?
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Correct Answer: B
Histology: Tumor composed of sheets of large polygonal cells with abundant granular eosinophilic cytoplasm.
Discussion: Granular cell epulis is a congenital lesion seen in the gingiva of newborn infants, almost exclusively girls. While similar to granular cell tumor, the granular cell epulis has a more prominent vasculature and does not induce psudohyperplasia of the overlying squamous epithelium. In contrast to granular cell tumor, granular cell epulis is negative for S100 by immunohistochemistry. The tumor is benign and recurrence is not common even when it is incompletely resected.
Histology: Tumor composed of sheets of large polygonal cells with abundant granular eosinophilic cytoplasm.
Discussion: Granular cell epulis is a congenital lesion seen in the gingiva of newborn infants, almost exclusively girls. While similar to granular cell tumor, the granular cell epulis has a more prominent vasculature and does not induce psudohyperplasia of the overlying squamous epithelium. In contrast to granular cell tumor, granular cell epulis is negative for S100 by immunohistochemistry. The tumor is benign and recurrence is not common even when it is incompletely resected.
Presented by Dr. Andres Matoso and prepared by Dr. Yembur Ahmad
46 year old female with an adrenal mass
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A 46 year old female presents with an adrenal mass. What is the diagnosis?
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Correct answer: D
Histology: This tumor is composed of nests and cords of highly atypical cells with eosinophilic cytoplasm replacing the entire adrenal parenchyma.
Discussion: The adrenal gland is one of the most common sites for metastatic carcinoma from lung, kidney, colon and involvement by melanoma and lymphoma. The differential diagnosis also includes primary adrenal lesions including adrenocortical neoplasia and pheochromocytomas. Adrenocortical carcinomas are rare while metastases are more common in the older patients. Immunohistochemistry can help in the differential diagnosis; adrenocortical neoplasias are usually negative or only focally positive for broad spectrum cytokeratins, while they are positive for inhibin, calretinin and SF1. Both pheochromocytomas and adrenocortical carcinomas can stain for synaptophysin, but staining for chromogranin makes pheochromocytoma more likely. Still, when expression of neuroendocrine markers is present, one should not forget that metastatic tumors can also express neuroendocrine markers, and therefore other organ specific markers should be included in the panel based on the morphologic features of the lesion and the past medical history of the patient.
Histology: This tumor is composed of nests and cords of highly atypical cells with eosinophilic cytoplasm replacing the entire adrenal parenchyma.
Discussion: The adrenal gland is one of the most common sites for metastatic carcinoma from lung, kidney, colon and involvement by melanoma and lymphoma. The differential diagnosis also includes primary adrenal lesions including adrenocortical neoplasia and pheochromocytomas. Adrenocortical carcinomas are rare while metastases are more common in the older patients. Immunohistochemistry can help in the differential diagnosis; adrenocortical neoplasias are usually negative or only focally positive for broad spectrum cytokeratins, while they are positive for inhibin, calretinin and SF1. Both pheochromocytomas and adrenocortical carcinomas can stain for synaptophysin, but staining for chromogranin makes pheochromocytoma more likely. Still, when expression of neuroendocrine markers is present, one should not forget that metastatic tumors can also express neuroendocrine markers, and therefore other organ specific markers should be included in the panel based on the morphologic features of the lesion and the past medical history of the patient.
Presented by Dr. Andres Matoso and prepared by Dr. Yembur Ahmad.
45 year old male with a renal mass.
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A 45 year old male presents with a renal mass.
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Correct answer: B
Histology: This tumor is composed by a dual population smaller cells surrounded by larger cells with clear or eosinophilic cytoplasm arrange in a nested pattern.
Discussion: 6;11 translocation RCC is a less common type of translocation RCC and has many overlapping morphologic and immunophenotypic features with TFE3 translocation RCC. The classic morphology of t(6;11) RCC is the dual population of smaller cells surrounded by larger cells arranged in a nested pattern as seen in this case. This case also shows prominent melanin pigment. By immunohistochemistry, these tumors are positive for cathepsin-K, HMB45 and Melan -A. While the majority of t(6;11) RCC have a favorable prognosis, recent studies with more aggressive clinical behavior have been reported.
Reference:
Am J Surg Pathol 2017;41:287-298
Am J Surg Pathol 2016;40:1484-1495
Incorrect
Correct answer: B
Histology: This tumor is composed by a dual population smaller cells surrounded by larger cells with clear or eosinophilic cytoplasm arrange in a nested pattern.
Discussion: 6;11 translocation RCC is a less common type of translocation RCC and has many overlapping morphologic and immunophenotypic features with TFE3 translocation RCC. The classic morphology of t(6;11) RCC is the dual population of smaller cells surrounded by larger cells arranged in a nested pattern as seen in this case. This case also shows prominent melanin pigment. By immunohistochemistry, these tumors are positive for cathepsin-K, HMB45 and Melan -A. While the majority of t(6;11) RCC have a favorable prognosis, recent studies with more aggressive clinical behavior have been reported.
Reference:
Am J Surg Pathol 2017;41:287-298
Am J Surg Pathol 2016;40:1484-1495
Presented by Dr. Andres Matoso and prepared by Dr. Yembur Ahmad.
53 year old female undergoing bilateral orchiectomy
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The images shown below are from a 53 year old female who has undergone a bilateral orchiectomy. What is your diagnosis?
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Correct answer: C
Histology: Testis with atrophic seminiferous tubules with appearance similar to Sertoli-only pattern, marked expansion of the intertubular space with peritubular fibrosis. Leydig cells are diminished in number.
Discussion: As tolerance to gender dysphoria increases in the United States, many more individuals will be opting for gender affirming surgery (GAS) and specimens that were once uncommon will begin to be more frequent in surgical pathology laboratories. The clinical care of persons undergoing GAS is standardized and all persons reaching the stage of the surgical procedure have undergone hormonal therapy aimed to achieve a feminine phenotype. The histologic findings in the testis associated with cross-hormone therapy in persons undergoing GAS. These include: 1) decreased diameter of seminiferous tubules and expansion of the interstitium; 2) marked hypoplasia of germ cells with aspermatogenesis; 3) rare cytomegaly; 4) hypoplasia or absence of Leydig cells; 5) epididymal hyperplasia of the epithelium and periepididymal fibrosis. This spectrum of findings can be summarized in the surgical pathology report as “changes consistent with cross-hormone therapy.”
Reference: Hum Pathol. 2018 Jun;76:91-99.
Incorrect
Correct answer: C
Histology: Testis with atrophic seminiferous tubules with appearance similar to Sertoli-only pattern, marked expansion of the intertubular space with peritubular fibrosis. Leydig cells are diminished in number.
Discussion: As tolerance to gender dysphoria increases in the United States, many more individuals will be opting for gender affirming surgery (GAS) and specimens that were once uncommon will begin to be more frequent in surgical pathology laboratories. The clinical care of persons undergoing GAS is standardized and all persons reaching the stage of the surgical procedure have undergone hormonal therapy aimed to achieve a feminine phenotype. The histologic findings in the testis associated with cross-hormone therapy in persons undergoing GAS. These include: 1) decreased diameter of seminiferous tubules and expansion of the interstitium; 2) marked hypoplasia of germ cells with aspermatogenesis; 3) rare cytomegaly; 4) hypoplasia or absence of Leydig cells; 5) epididymal hyperplasia of the epithelium and periepididymal fibrosis. This spectrum of findings can be summarized in the surgical pathology report as “changes consistent with cross-hormone therapy.”
Presented by Dr. Andres Matoso and prepared by Dr. Yembur Ahmad
Case 1: A 6 year old boy with a testicular mass.
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A 6 year old boy presents with a testicular mass. Choose the correct diagnosis.
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Correct answer: B
Histology: The tumor is composed by a cyst lined by well-differentiated squamous epithelium without adnexa. The surrounding testis is normal for the patient’s age and does not show active spermatogenesis or germ cell neoplasia in-situ.
Discussion: Epidermoid cyst occur in males up to 40 years of age. In contrast to malignant teratomas, the epidermoid cyst is composed of only one type of epithelium (keratinizing squamous) and it is not surrounded by a cellular stroma that characterizes the malignant teratomas. The surrounding testis is normal, while in malignant teratoma there usually is associated germ cell neoplasia in-situ. Occasional rupture of epidermoid inclusion cysts can lead to a giant cell reaction.
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Correct answer: B
Histology: The tumor is composed by a cyst lined by well-differentiated squamous epithelium without adnexa. The surrounding testis is normal for the patient’s age and does not show active spermatogenesis or germ cell neoplasia in-situ.
Discussion: Epidermoid cyst occur in males up to 40 years of age. In contrast to malignant teratomas, the epidermoid cyst is composed of only one type of epithelium (keratinizing squamous) and it is not surrounded by a cellular stroma that characterizes the malignant teratomas. The surrounding testis is normal, while in malignant teratoma there usually is associated germ cell neoplasia in-situ. Occasional rupture of epidermoid inclusion cysts can lead to a giant cell reaction.
Presented by Dr. Pedram Argani and prepared by Dr. Yembur Ahmad.
1 year old male with a retroperitoneal mass.
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This is a 1 year old male with a retroperitoneal mass. What is the diagnosis?
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Answer: C
Histologic Description: This is a fatty lesion that has a variety of histologic appearances. Toward the edge of the lesion, the lesion is more cellular, and primitive fat cells are interspersed with a branching capillary vasculature that suggests developing fat or myxoid liposarcoma. Adjacent to these areas are myxoid pools, which again suggests myxoid liposarcoma. Toward the central part of the lesion are more differentiated fat cells that closely resemble normal fat. This neoplasm demonstrated rearrangement of the PLAG1 gene and an absence of rearrangement of the DDIT3 gene, supporting the diagnosis of lipoblastoma. On clinical grounds, the patient’s age also strongly supports lipoblastoma.
Differential Diagnosis: Well-differentiated liposarcoma typically occurs in adults, and features highly pleomorphic lipoblasts that are centered on fibrous septa in a background of differentiated mature-appearing fat. Myxoid liposarcoma would lack the zonation of the current lesion, typically occurs in older patients, and demonstrates the characteristic FUS-DDIT3 gene fusion that is not seen in lipoblastoma. Fat necrosis typically does not demonstrate the myxoid appearance seen in the current case, and has prominent histiocytes.
90% of cases of lipoblastoma occur in patients under the age of 5. Approximately 1/6th of patients have an associated disorder of the central nervous system. The zonal maturation of the lesions (immature myxoid cells at the periphery, mature fat centrally) recapitulates fetal fat development.
Incorrect
Answer: C
Histologic Description: This is a fatty lesion that has a variety of histologic appearances. Toward the edge of the lesion, the lesion is more cellular, and primitive fat cells are interspersed with a branching capillary vasculature that suggests developing fat or myxoid liposarcoma. Adjacent to these areas are myxoid pools, which again suggests myxoid liposarcoma. Toward the central part of the lesion are more differentiated fat cells that closely resemble normal fat. This neoplasm demonstrated rearrangement of the PLAG1 gene and an absence of rearrangement of the DDIT3 gene, supporting the diagnosis of lipoblastoma. On clinical grounds, the patient’s age also strongly supports lipoblastoma.
Differential Diagnosis: Well-differentiated liposarcoma typically occurs in adults, and features highly pleomorphic lipoblasts that are centered on fibrous septa in a background of differentiated mature-appearing fat. Myxoid liposarcoma would lack the zonation of the current lesion, typically occurs in older patients, and demonstrates the characteristic FUS-DDIT3 gene fusion that is not seen in lipoblastoma. Fat necrosis typically does not demonstrate the myxoid appearance seen in the current case, and has prominent histiocytes.
90% of cases of lipoblastoma occur in patients under the age of 5. Approximately 1/6th of patients have an associated disorder of the central nervous system. The zonal maturation of the lesions (immature myxoid cells at the periphery, mature fat centrally) recapitulates fetal fat development.
Presented by Dr. Pedram Argani and prepared by Dr. Yembur Ahmad.
55 year old female with an esophageal mass.
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This is a 55 year old female with an esophageal mass. What is the diagnosis?
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Answer: C
Histologic Description: This is solid neoplasm composed of spindle to epithelioid cells with prominent granular cytoplasm. Cytoplasmic granules are predominantly small, but occasional granules are distinctively larger. The neoplasm labeled for S100 protein and inhibin, supporting the diagnosis.
Differential Diagnosis: Smooth muscle neoplasms such as leiomyoma and leiomyosarcoma would have better defined, less granular cytoplasm that would label with desmin. Schwannomas would label for S100 protein, but characteristically demonstrate hyaline vessels, angulated nuclei with clear cytoplasm, palisading of nuclei (Verocay bodies), and alternations between cellular and hypocellular areas. Schwannomas typically do not label for inhibin. Granular cell tumors are typically treated by complete excision. Most are benign, but rare benign appearing lesions can metastasize. Histologic risk factors include necrosis, prominent spindling, prominent nucleoli, high mitotic rate (>2 per 10 mitoses per high power field), pleomorphism and high nucleus/cytoplasm ratio. None of these features are present in the current case.
Incorrect
Answer: C
Histologic Description: This is solid neoplasm composed of spindle to epithelioid cells with prominent granular cytoplasm. Cytoplasmic granules are predominantly small, but occasional granules are distinctively larger. The neoplasm labeled for S100 protein and inhibin, supporting the diagnosis.
Differential Diagnosis: Smooth muscle neoplasms such as leiomyoma and leiomyosarcoma would have better defined, less granular cytoplasm that would label with desmin. Schwannomas would label for S100 protein, but characteristically demonstrate hyaline vessels, angulated nuclei with clear cytoplasm, palisading of nuclei (Verocay bodies), and alternations between cellular and hypocellular areas. Schwannomas typically do not label for inhibin. Granular cell tumors are typically treated by complete excision. Most are benign, but rare benign appearing lesions can metastasize. Histologic risk factors include necrosis, prominent spindling, prominent nucleoli, high mitotic rate (>2 per 10 mitoses per high power field), pleomorphism and high nucleus/cytoplasm ratio. None of these features are present in the current case.
Presented by Dr. Pedram Argani and prepared by Dr. Yembur Ahmad.
61 year old male with a renal mass.
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This is a 61 year old male with a renal mass. The patient has been on dialysis. What is the diagnosis?
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Answer: B
Histologic Description: This renal neoplasm arises in an end stage kidney. It is associated with extensive cystic change and hemorrhage. The epithelial component of the lesion has a cribriform, sieve-like architecture. The cribriform architecture results from intracellular and intercellular spaces. The neoplastic cells are epithelioid and have abundant pink cytoplasm and round nuclei with occasional prominent nucleoli. Interspersed between the neoplastic cells are calcium oxalate crystals which are refractile and polarizable. These are the characteristic features of acquired cystic disease associated renal cell carcinoma (ACD-RCC).
Differential Diagnosis: Eosinophilic solid and cystic renal cell carcinoma (ESC-RCC) features epithelioid eosinophilic cells, but the cells demonstrate prominent basophilic cytoplasmic precipitates and lack oxalate crystals. Succinate dehydratase deficient renal cell carcinomas characteristically have intracytoplasmic vacuoles and inclusions, and lack oxalate crystals. These neoplasms demonstrate loss of SDHB immunoreactivity. Fumarate hydratase deficient renal cell carcinomas characteristically demonstrate vesicular chromatin with prominent nucleoli, and often have papillary or solid appearance. These neoplasms lack oxalate crystals, and demonstrate loss of fumarate hydratase immunoreactivity.
Incorrect
Answer: B
Histologic Description: This renal neoplasm arises in an end stage kidney. It is associated with extensive cystic change and hemorrhage. The epithelial component of the lesion has a cribriform, sieve-like architecture. The cribriform architecture results from intracellular and intercellular spaces. The neoplastic cells are epithelioid and have abundant pink cytoplasm and round nuclei with occasional prominent nucleoli. Interspersed between the neoplastic cells are calcium oxalate crystals which are refractile and polarizable. These are the characteristic features of acquired cystic disease associated renal cell carcinoma (ACD-RCC).
Differential Diagnosis: Eosinophilic solid and cystic renal cell carcinoma (ESC-RCC) features epithelioid eosinophilic cells, but the cells demonstrate prominent basophilic cytoplasmic precipitates and lack oxalate crystals. Succinate dehydratase deficient renal cell carcinomas characteristically have intracytoplasmic vacuoles and inclusions, and lack oxalate crystals. These neoplasms demonstrate loss of SDHB immunoreactivity. Fumarate hydratase deficient renal cell carcinomas characteristically demonstrate vesicular chromatin with prominent nucleoli, and often have papillary or solid appearance. These neoplasms lack oxalate crystals, and demonstrate loss of fumarate hydratase immunoreactivity.
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