Summary

Renal cell carcinoma (RCC), the most common malignancy of kidney, originates from renal tubular epithelium. It is subclassified based on histological and molecular features. Rarely, RCC can show focal to extensive sarcomatoid or rhabdoid differentiation. RCC with extensive sarcomatoid differentiation and no identifiable epithelial component is designated as unclassified RCC with sarcomatoid differentiation. Presence of sarcomatoid or rhabdoid differentiation is associated with poor prognosis. We describe autopsy findings in a case of RCC with extensive sarcomatoid and focal rhabdoid differentiation presenting with malignant ascites secondary to peritoneal carcinomatosis and multiorgan metastasis.