Abstract

Succinate dehydrogenase (SDH)-deficient gastrointestinal stromal tumors (GIST) are a rare subset of GIST that mostly affect adolescents and young adults. SDH-deficient GISTs have the propensity to frequently metastasize to the liver. Because these tumors lack mutations in the proto-oncogene receptor tyrosine kinase, KIT and PDGFRA, they generally do not respond well to standard treatments for GIST. Surgical management is considered first-line treatment; however, due to the presence of often diffuse, bilobar liver metastases, hepatic resection is often not feasible.

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