Dr. Linehan on Treatment for Hereditary Leiomyomatosis and RCC

W. Marston Linehan, MD, chief, Urologic Oncology Branch, National Cancer Institute, discusses the treatment of hereditary leiomyomatosis and renal cell cancer (HLRCC).

HLRCC is hereditary cancer syndrome in which patients are at risk to develop cutaneous leiomyomas, uterine leiomyomas, and eventually a very aggressive form of type 2 papillary kidney cancer.

This is one of the most aggressive forms of kidney cancer, says Linehan, and recommended treatment is usually immediate surgical intervention. But, efforts are being focused on developing a therapy that targets the pathway of the Krebs cycle enzyme gene fumarate hydratase. One example is the combination of bevacizumab (Avastin) and erlotinib (Tarceva), which is demonstrating dramatic, prolonged responses.