Fumarate Hydratase-Deficient Renal Cell Carcinoma

**Semantic type:** Neoplastic Process

**Definition:** A rare, highly aggressive renal cell carcinoma caused by germline or (in a subset) somatic mutation of fumarate hydratase gene. It arises mostly in the setting of hereditary leiomyomatosis-renal cell carcinoma syndrome.

**Synonyms:** - FH-Deficient RCC - FH-Deficient Renal Cell Carcinoma - Fumarate Hydratase Deficient Renal Cell Carcinoma