TITLE:
Succinate Dehydrogenase-Deficient Renal Cell Carcinoma: A Case Report and Brief Review of the Literature—SDH-Deficient RCC
AUTHORS:
Selin Kestel, Ahmet Olgun, Fazlı Polat, Ali Atan, Serhat Cetin, Ipek Isik Gonul, Ozgur Ekinci
KEYWORDS:
Succinate Dehydrogenase Deficiency, Renal Cell Carcinoma, SDHB, SDHA
JOURNAL NAME:
Case Reports in Clinical Medicine,
Vol.15 No.10,
October
21,
2026
ABSTRACT: Succinate dehydrogenase (SDH) is a critical enzyme that connects the tricarboxylic acid cycle and the mitochondrial electron transport chain, and pathogenic germline variants in SDH-related genes may be inherited in an autosomal dominant pattern, unlike other mitochondrial complexes, and predispose to SDH-deficient RCC. A 24-year-old male presenting with hematuria was found to have a mass in the left kidney. A radical nephrectomy was performed. Gross examination showed an exophytic mass at the upper pole of the left kidney. The tumor was well-circumscribed with a tan-brown, red-cut surface. Microscopically, the tumor had a pushing border, entrapping benign tubules at the periphery. The neoplastic cells demonstrated a variably solid or nested architecture and had eosinophilic to clear wispy cytoplasmic vacuoles or inclusions representing the large mitochondria. Immunohistochemically, complete loss of expression for SDH-B in the tumor cells was evident. SDH-deficient renal cell carcinoma is described as a rare entity with fewer than 75 case reports.