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A recent study, published in Cancer Research and Treatment in March 2019, provides the first real-world evidence of the promising treatment of advanced hereditary leiomyomatosis and renal cell carcinoma (HLRCC), a rare genetic syndrome resulting from mutations in the gene that makes an enzyme called fumarate hydratase.
Ten patients with advanced HLRCC-associated renal cell carcinoma (RCC) were identified in Korea. In these patients, the overall response rate to treatment with a combination of bevacizumab plus erlotinib was 50% and median progression-free survival and overall survival were 13.3 and 14.1 months, respectively.
The researchers concluded that the combination of bevacizumab plus erlotinib showed promising activity with moderate toxicity in patients with HLRCC-associated RCC, and recommend bevacizumab plus erlotinib as a first-line treatment for this rare genetic disorder.