Case History
Answer:
D. Steroid cell tumor, NOS
Steroid cell tumor NOS is the correct diagnosis. The tumor cells are SF-1+, inhibin +, calretinin+, EMA-, PAX8-, and CAIX-. All of the other and choices would be in the differential. Leydig cell tumors have an identical immunoprofile, but Reinke crystals would be present and are required for the diagnosis. Clear cell carcinoma of the ovary can form solid groups of clear cells, but the cells would be EMA+ and PAX8+ and negative for the typical sex cord tumor markers. Metastatic clear cell renal cell carcinoma may show similar clear cell morphology, but would have a different immunoprofile including being PAX8+, CAIX+, and EMA+. Metastatic adrenal cortical carcinoma would also be a consideration if there was history of an adrenal mass. The immunoprofile would be essentially the same.
Case contributed by: Tyler Clemmensen, M.D., UAB Pathology GYN/GU Pathology Fellow