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Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen.
This is a 50 year old female with a renal mass.
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Answer: D
Histologic Description: This is a solid lesion with a predominant tubular pattern. Much of the lesion lacks significantly prominent nucleoli, and the round nuclei raise the differential diagnosis of oncocytoma. The neoplasm demonstrates loss of fumarate hydrates by immunohistochemistry, supporting classification as fumarate hydratase deficient renal cell carcinoma.
Differential Diagnosis: Oncocytoma would feature perfectly round nuclei and a more prominent edematous stroma. Chromophobe renal cell carcinoma would have a more sheet like growth pattern, hyalinized vessels, and irregular nuclear contours. Eosinophilic solid and cystic renal carcinoma features polygonal eosinophilic cells with basophilic cytoplasmic stippling, and typically demonstrates patchy immunoreactivity for cytokeratin 20. It is associated with somatic mutations in the TSC1 or TSC2 genes.
Fumarate hydratase deficient renal cell carcinomas are hereditary in approximately 80% of cases and sporadic in the remaining 20%. The typical morphology is that of a high grade “type II” papillary renal cell carcinoma pattern with extremely prominent cherry red nucleoli. However, the morphologic spectrum is broad and includes cysts and oncocytoma-like lesions such as the current case.Incorrect
Answer: D
Histologic Description: This is a solid lesion with a predominant tubular pattern. Much of the lesion lacks significantly prominent nucleoli, and the round nuclei raise the differential diagnosis of oncocytoma. The neoplasm demonstrates loss of fumarate hydrates by immunohistochemistry, supporting classification as fumarate hydratase deficient renal cell carcinoma.
Differential Diagnosis: Oncocytoma would feature perfectly round nuclei and a more prominent edematous stroma. Chromophobe renal cell carcinoma would have a more sheet like growth pattern, hyalinized vessels, and irregular nuclear contours. Eosinophilic solid and cystic renal carcinoma features polygonal eosinophilic cells with basophilic cytoplasmic stippling, and typically demonstrates patchy immunoreactivity for cytokeratin 20. It is associated with somatic mutations in the TSC1 or TSC2 genes.
Fumarate hydratase deficient renal cell carcinomas are hereditary in approximately 80% of cases and sporadic in the remaining 20%. The typical morphology is that of a high grade “type II” papillary renal cell carcinoma pattern with extremely prominent cherry red nucleoli. However, the morphologic spectrum is broad and includes cysts and oncocytoma-like lesions such as the current case.