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Presented by Jonathan Epstein, M.D. and prepared by Doreen Nguyen, M.D.
Case 1: An 85-year-old male underwent a resection of a cystic kidney tumor.
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Question 1 of 1
1. Question
Week 551: Case 1
An 85-year-old male underwent a resection of a cystic kidney tumor.
Correct
Answer: Tubulocystic carcinoma
Histology: The lesion consists of multiple cysts of varying shapes. Composed of large, round-to-polygonal cells with abundant eosinophilic cytoplasm. Cysts are lined by moderately atypical epithelial cells with vesicular nuclei and prominent nucleoli. Nuclei vary in shape and some have irregular shapes.
Discussion: Tubulocystic carcinomas arise in adults with a mean age at diagnosis of 57 years (30-80 years). It is male predominant. The tumor is unifocal with predominantly cortical location. There are conflicting studies on the relationship between tubulocystic carcinomas and papillary RCC. Focal areas of papillary RCC can be seen in tubulocystic carcinoma. The tumors are well-circumscribed but not encapsulated. They average 4.3 cm. (0.5-17.5 cm.). Necrosis and hemorrhage are not seen. Typical multilocular cystic cut surface impart a unique sponge-like appearance. Cystic nephroma would have either flattened or hobnail epithelial lining with bland nuclei. Also, cystic nephroma typically occurs in females and has ovarian-like stroma in the wall. Multicystic RCC would have low grade renal cell carcinoma in the cyst wall. Oncocytomas can uncommonly be composed of tubules with dilatation mimicking tubulocystic RCC. However, the nuclei in oncocytomas lack the variation in size and shape seen in tubulocystic RCC.
The prognosis for tubulocystic RCC is favorable with less that 10% rate of reported metastases. These tumors are not assigned a Fuhrman grade.
Incorrect
Answer: Tubulocystic carcinoma
Histology: The lesion consists of multiple cysts of varying shapes. Composed of large, round-to-polygonal cells with abundant eosinophilic cytoplasm. Cysts are lined by moderately atypical epithelial cells with vesicular nuclei and prominent nucleoli. Nuclei vary in shape and some have irregular shapes.
Discussion: Tubulocystic carcinomas arise in adults with a mean age at diagnosis of 57 years (30-80 years). It is male predominant. The tumor is unifocal with predominantly cortical location. There are conflicting studies on the relationship between tubulocystic carcinomas and papillary RCC. Focal areas of papillary RCC can be seen in tubulocystic carcinoma. The tumors are well-circumscribed but not encapsulated. They average 4.3 cm. (0.5-17.5 cm.). Necrosis and hemorrhage are not seen. Typical multilocular cystic cut surface impart a unique sponge-like appearance. Cystic nephroma would have either flattened or hobnail epithelial lining with bland nuclei. Also, cystic nephroma typically occurs in females and has ovarian-like stroma in the wall. Multicystic RCC would have low grade renal cell carcinoma in the cyst wall. Oncocytomas can uncommonly be composed of tubules with dilatation mimicking tubulocystic RCC. However, the nuclei in oncocytomas lack the variation in size and shape seen in tubulocystic RCC.
The prognosis for tubulocystic RCC is favorable with less that 10% rate of reported metastases. These tumors are not assigned a Fuhrman grade.
