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Presented by Dr. Pedram Argani and prepared by Dr. Jennifer Bynum
This is an adrenal tumor in a 41 year old male with a history of multifocal clear cell renal cell carcinoma.
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1. Question
Week 630: Case 1
This is an adrenal tumor in a 41 year old male with a history of multifocal clear cell renal cell carcinoma.
Correct
Answer: A
Histologic Description: This is a highly vascular clear cell neoplasm in the adrenal, which certainly raises the differential diagnosis of clear cell RCC. However, the neoplastic cells in this case also have finely granular amphophilic cytoplasm in different areas, and irregular, coarse chromatin. This features support the diagnosis of pheochromocytoma. The neoplasm was immunoreactive for chromogranin and synaptophysin, but not for PAX8, supporting the above diagnosis.
Differential Diagnosis: Adrenal cortical neoplasms would have more vacuolated cytoplasm, with vacuoles that indent the nucleus as is of typical steroid-producing cellular lesions. These neoplasms would not label for chromogranin. Metastatic renal cell carcinoma would lack the amphophilic fine cytoplasmic granules in the current case, would typically not label for chromogranin and synaptophysin, and would label for PAX8.
This patient likely had von Hippel Lindau syndrome, which predisposes two multiple clear cell RCCs and pheochromocytoma.
Incorrect
Answer: A
Histologic Description: This is a highly vascular clear cell neoplasm in the adrenal, which certainly raises the differential diagnosis of clear cell RCC. However, the neoplastic cells in this case also have finely granular amphophilic cytoplasm in different areas, and irregular, coarse chromatin. This features support the diagnosis of pheochromocytoma. The neoplasm was immunoreactive for chromogranin and synaptophysin, but not for PAX8, supporting the above diagnosis.
Differential Diagnosis: Adrenal cortical neoplasms would have more vacuolated cytoplasm, with vacuoles that indent the nucleus as is of typical steroid-producing cellular lesions. These neoplasms would not label for chromogranin. Metastatic renal cell carcinoma would lack the amphophilic fine cytoplasmic granules in the current case, would typically not label for chromogranin and synaptophysin, and would label for PAX8.
This patient likely had von Hippel Lindau syndrome, which predisposes two multiple clear cell RCCs and pheochromocytoma.
