Treatment Selection in FH-Deficient Renal Cell Carcinoma

Dr. Voss and Dr. Serzan focus on treatment considerations for patients with FH-deficient renal cell carcinoma (RCC), highlighting emerging evidence for distinct therapeutic approaches in this molecularly defined disease. Dr. Serzan reviews NIH data evaluating bevacizumab plus erlotinib in patients with papillary and FH-deficient RCC, noting the reported response activity, progression-free survival, and generally manageable toxicity profile. The discussion then compares this approach with cabozantinib plus nivolumab, based on prospective phase 2 data showing encouraging activity in FH-deficient RCC. Dr. Serzan describes his preference for cabozantinib plus nivolumab in the first-line setting, while Dr. Voss similarly favors this approach and discusses how evolving molecular classification and genomic analyses have increased recognition of FH-deficient RCC as a distinct disease entity. The experts also consider treatment sequencing, noting that the two regimens have different mechanisms of action and have both been studied in first- and later-line settings. They discuss the potential role of using the alternative regimen following progression, while emphasizing the limited evidence base for optimal sequencing in this rare RCC subtype.