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Presented by Jonathan Epstein, M.D. and prepared by Sarah Karram, M.D.
A 55 year old man with renal failure underwent a nephrectomy.
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1. Question
Week (612): Case 3
A 55 year old man with renal failure underwent a nephrectomy.
Correct
Answer: Acquired cystic kidney disease associated renal cell carcinoma
Histology: The tumor is composed of cells with abundant eosinophilic cytoplasm arranged in a cribriform pattern. Oxylate crystals are present. The surrounding kidney shows end stage renal disease (ESRD).
Discussion: Patients with ESRD have an increased risk of developing renal cell carcinoma. While many different tumor variants can occur, the one tumor that exclusively occurs in this setting is acquired kidney disease (ACKD) associated RCC. The morphology of these tumors are consistent from one case to another, and identical to the current case. The cribriform pattern is also referred to as “sieve-like”. One can also see in the adjacent non-neoplastic kidney cysts that are lined by similar epithelium either as a single cell lining or with minimal cribriform growth, yet lacking the solid growth of ACRD associated RCC. These atypical cysts are thought to be precursor lesions to these distinctive carcinomas. Cribriform growth is uncommon in renal cell carcinoma and renal medullary carcinoma is the other variant with this morphology. Renal medullary carcinoma occurs in the setting of sickle cell trait and other than the cribriform growth pattern has a different morphology. ACKD associated RCC has an intermediate prognosis and is worse than clear cell papillary RCC, which is one of the other common tumors to arise in the setting of ESRD.
Incorrect
Answer: Acquired cystic kidney disease associated renal cell carcinoma
Histology: The tumor is composed of cells with abundant eosinophilic cytoplasm arranged in a cribriform pattern. Oxylate crystals are present. The surrounding kidney shows end stage renal disease (ESRD).
Discussion: Patients with ESRD have an increased risk of developing renal cell carcinoma. While many different tumor variants can occur, the one tumor that exclusively occurs in this setting is acquired kidney disease (ACKD) associated RCC. The morphology of these tumors are consistent from one case to another, and identical to the current case. The cribriform pattern is also referred to as “sieve-like”. One can also see in the adjacent non-neoplastic kidney cysts that are lined by similar epithelium either as a single cell lining or with minimal cribriform growth, yet lacking the solid growth of ACRD associated RCC. These atypical cysts are thought to be precursor lesions to these distinctive carcinomas. Cribriform growth is uncommon in renal cell carcinoma and renal medullary carcinoma is the other variant with this morphology. Renal medullary carcinoma occurs in the setting of sickle cell trait and other than the cribriform growth pattern has a different morphology. ACKD associated RCC has an intermediate prognosis and is worse than clear cell papillary RCC, which is one of the other common tumors to arise in the setting of ESRD.
