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Clear renal cell carcinoma with angioleiomyomatous stroma in a noninherited renal cancer syndrome: Is it a different entity?

Renal cell carcinoma (RCC) comprises only 3% of adult cancer cases. In most cases, patient is asymptomatic and the tumor mass is incidentally detected during radiological imaging for other medical conditions. Only one-third of patients with RCC present with hematuria, palpable tumor mass, or flank pain. Most of the cases of RCC are with clear or papillary cells, but a small subset of cases have demonstrated clear cell RCC with leiomyomatous stroma. The World Health Organization classification of tumors of the urinary system and male genital organs 2016 and the International Society Urological Pathology 2012 Vancouver classification have identified RCC with leiomyomatous stroma (RCCLMS) as an emerging entity of RCC. RCCLMS has been seen with inherited renal cancer syndromes such as tuberous sclerosis complex and von Hippel-Lindau disease. Here, we present a rare case of RCC with morphological and histopathological findings of the RCCLMS subset but showed noninherited mutational traits.