Succinate dehydrogenase-deficient renal cell carcinoma


  • A variant of renal cell carcinoma (RCC) defined by the loss of protein expression of succinate dehydrogenase B on immunohistochemistry

Clinical features


  • Rare, <0.2% of all RCCs

  • Mean age, 38 years (range, 14–76)

  • Male to female ratio, 1.8:1

  • Majority of cases occur in the setting of germline mutations in one of the succinate dehydrogenase (SDH) genes, most commonly SDHB

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