ArticleClinical cancer research : an official journal of the American Association for Cancer Research2021
Comprehensive Molecular Characterization and Response to Therapy in Fumarate Hydratase-Deficient Renal Cell Carcinoma.
Article in Clinical cancer research : an official journal of the American Association for Cancer Research, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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Who cites it
40 citing papers in PubMed, 85 citations in OpenAlex.
- Tislelizumab plus lenvatinib in advanced fumarate hydratase-deficient renal cell carcinoma: a single-arm, phase 2 trial with metabolic biomarker analysis.Nature communications · 2026Trial
- Sintilimab Plus Axitinib for Advanced Fumarate Hydratase-Deficient Renal Cell Carcinoma: A Phase 2 Nonrandomized Clinical Trial.JAMA oncology · 2025Trial
- A Phase 2 Trial of Talazoparib and Avelumab in Genomically Defined Metastatic Kidney Cancer.European urology oncology · 2024Trial
- Phase II Trial of Cabozantinib Plus Nivolumab in Patients With Non-Clear-Cell Renal Cell Carcinoma and Genomic Correlates.Journal of clinical oncology : official journal of the American Society of Clinical Oncology · 2022Trial
- HIRA loss is frequent but not universal in human fumarate hydratase-deficient renal cell carcinoma and defines divergent oncogenic programs.Biomarker research · 2026Article
- Expansion of Germline Variants in Primary Hyperparathyroidism: Fumarate Hydratase Deficiency as a Cause of Parathyroid Adenomas.Endocrine pathology · 2026Observational
- Case Report: A patient with metastatic fumarate hydratase-deficient renal cell carcinoma associated with leiomyomatosis: real-world clinical insights on systemic therapy and liver-directed SBRT.Frontiers in oncology · 2026Article
- Differential Efficacy of Bevacizumab and Erlotinib in Preclinical Models of Renal Medullary Carcinoma and Fumarate Hydratase-Deficient Renal Cell Carcinoma.Molecular cancer therapeutics · 2025Article
- Stimulator of interferon genes (STING) immunohistochemical expression in fumarate hydratase-deficient renal cell carcinoma: biological and potential predictive implications.Virchows Archiv : an international journal of pathology · 2025Article
- Evolving trends and clinical-pathological correlations in renal cell carcinoma surgery: a decade-long study at Peking University First Hospital.Clinical and experimental medicine · 2025Article
- Targeting metabolic and epigenetic reprogramming in metastatic fumarate hydratase-deficient renal cell carcinoma.Clinical & experimental metastasis · 2025Review
- Clinicopathologic and prognosis of 30 patients with FH-deficient renal cell carcinoma: a single-center retrospective study.BMC cancer · 2025Article
- Metabolic tumor parameters onAnnals of nuclear medicine · 2025Article
- Comprehensive molecular profiling of FH-deficient renal cell carcinoma identifies molecular subtypes and potential therapeutic targets.Nature communications · 2025Article
- Morphological analysis of peritoneal fluid in fumarate hydratase deficiency-associated renal cell carcinoma: a case report.Frontiers in oncology · 2025Article
- Fumarate hydratase-deficient renal cell carcinoma with sarcomatoid features: a case report.AME case reports · 2025Article
- Integrated multi-omics analysis reveals a glycolytic signature that predicts pan-cancer immune checkpoint inhibitor response and LDHA as a combinatorial target in fumarate hydratase-deficient renal cell carcinoma.Frontiers in immunology · 2025Article
- Non-clear cell renal cell carcinoma narrative review: where we are in 2024.Translational cancer research · 2024Review
- Tricarboxylic Acid Cycle Relationships with Non-Metabolic Processes: A Short Story with DNA Repair and Its Consequences on Cancer Therapy Resistance.International journal of molecular sciences · 2024Review
- Case report: response to immunotherapy and association with the fh gene in hereditary leiomyomatosis and renal cell cancer-associated renal cell cancer.BMC medical genomics · 2024Article
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21 authors at 2 institutions in 1 country.
Funding
Abstract
purposeFumarate 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
designWe studied clinical and genomic characteristics of FH-RCC, including response [objective response rate (ORR)] to systemic therapies and next-generation sequencing (NGS). Patients with metastatic FH-RCC, defined by presence of pathogenic germline or somatic
resultsA total of 28 of 32 included patients (median age 46; range, 20-74; M:F, 20:12) underwent germline testing; 23 (82%) harbored a pathogenic
conclusionsAlthough most FH-RCC tumors are due to germline
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