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Presented by Jonathan Epstein, M.D. and prepared by Doreen Nguyen, M.D.
Case 3: A 70-year-old female underwent a nephrectomy for a cystic mass.
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1. Question
Week 553: Case 3
A 70-year-old female underwent a nephrectomy for a cystic mass.
Correct
Answer: A and B
Histology: The lesion consists of variable sized cysts lined by flattened to cuboidal clear cells. Occasional septa contain small aggregates of polygonal epithelial cells with typical features of clear cell renal cell carcinoma. There is optically clear cytoplasm, distinct cell borders and round to ovoid nuclei (Fuhrman grade 1).
Discussion: The major differential is RCC with cystic change. In multicystic RCC of LMP, the solid component limited to small sized non-expansile yellow grey areas within septa. In RCC with cystic change, the septae and wall of cysts contain sheets of polygonal cells with expansile solid appearance. Surgical removal is curative. Cases with >95% cyst formation and rare non-expansile low grade clear cell nests in the septae have never been shown to exhibit malignant behavior and it would be hard to imagine how they could do so. We diagnose these lesions as “multicystic renal cell neoplasm of low malignant potential tumors” rather than benign given the lack of long-term follow-up data on these cases. On the other hand we avoid labeling the patient as having “carcinoma” which has psychosocial and financial implications. Others use the term “multicystic renal cell carcinoma” for these lesions. In cases with >20% solid areas, uniformly accepted as cystic carcinomas. Unclear in the absence of data the malignant potential of 5%-20% solid areas but the current convention is to diagnose them as RCC with cystic change with a comment that their prognosis is favorable.
Incorrect
Answer: A and B
Histology: The lesion consists of variable sized cysts lined by flattened to cuboidal clear cells. Occasional septa contain small aggregates of polygonal epithelial cells with typical features of clear cell renal cell carcinoma. There is optically clear cytoplasm, distinct cell borders and round to ovoid nuclei (Fuhrman grade 1).
Discussion: The major differential is RCC with cystic change. In multicystic RCC of LMP, the solid component limited to small sized non-expansile yellow grey areas within septa. In RCC with cystic change, the septae and wall of cysts contain sheets of polygonal cells with expansile solid appearance. Surgical removal is curative. Cases with >95% cyst formation and rare non-expansile low grade clear cell nests in the septae have never been shown to exhibit malignant behavior and it would be hard to imagine how they could do so. We diagnose these lesions as “multicystic renal cell neoplasm of low malignant potential tumors” rather than benign given the lack of long-term follow-up data on these cases. On the other hand we avoid labeling the patient as having “carcinoma” which has psychosocial and financial implications. Others use the term “multicystic renal cell carcinoma” for these lesions. In cases with >20% solid areas, uniformly accepted as cystic carcinomas. Unclear in the absence of data the malignant potential of 5%-20% solid areas but the current convention is to diagnose them as RCC with cystic change with a comment that their prognosis is favorable.
