Comprehensive Molecular Characterization and Response to Therapy in Fumarate Hydratase-Deficient Renal Cell Carcinoma.

doi:10.1158/1078-0432.CCR-20-4367

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Article

Comprehensive Molecular Characterization and Response to Therapy in Fumarate Hydratase-Deficient Renal Cell Carcinoma.

Clinical cancer research : an official journal of the American Association for Cancer Research - 15 May 2021

Gleeson Jack P, Nikolovski Ines, Dinatale Renzo, Zucker Mark, Knezevic Andrea, Patil Sujata, Ged Yasser, Kotecha Ritesh R, Shapnik Natalie, Murray Samuel, Russo Paul, Coleman Jonathan, Lee Chung Han, Stadler Zsofia K, Hakimi A Ari, Feldman Darren R, Motzer Robert J, Reznik Ed, Voss Martin H, Chen Ying-Bei, Carlo Maria I

Abstract excerpt

PURPOSE: Fumarate hydratase-deficient renal cell carcinoma (FH-RCC) is a rare, aggressive form of RCC associated with hereditary leiomyomatosis and RCC syndrome. Evidence for systemic therapy efficacy is lacking. EXPERIMENTAL DESIGN: We studied clinical and genomic characteristics of FH-RCC, including response [objective response rate (ORR)] to systemic therapies and next-generation sequencing (NGS). Patients...

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PMID: 33658299
DOI: 10.1158/1078-0432.CCR-20-4367
PMCID: PMC8127353

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Source receipt: pubmed, retrieved 7 Sept 2026.

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