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In this video, Dr W. Marston Linehan, chief of the Urologic Oncology Branch of the National Cancer Institute in America, discusses the treatment of hereditary leiomyomatosis and renal cell carcinoma (HLRCC).
HLRCC is very rare inherited condition, in which patients are at risk of developing non-cancerous (benign) tumours in the muscle under the skin (cutaneous leiomyomas), in the womb of women (uterine leiomyomas), and eventually a very aggressive form of type 2 papillary kidney cancer (type 2 pRCC). HLRCC is caused by a change (mutation) in a gene called the fumarate hydratase (FH) gene. Type 2 pRCC is one of the most aggressive forms of kidney cancer, and the first-line of treatment is immediate removal of the tumour.
Research is focused on developing a drug treatment that targets the fumarate hydratase pathway in the Krebs cycle, the means by which cells release energy from food. One example is the combination of bevacizumab (Avastin) and erlotinib (Tarceva), which is demonstrating dramatic, prolonged responses.