SMARCB1-Deficient Kidney Medullary Carcinoma

**Semantic type:** Neoplastic Process

**Definition:** A high-grade carcinoma that arises from the renal medulla and is characterized by inactivation of the SMARCB1 gene. It affects children and adults and occurs mainly in patients with sickle cell trait. The majority of the cases occur in the right kidney.

**Synonyms:** - SMARCB1-Deficient Renal Medullary Carcinoma