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.

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Dr. Linehan on Treatment for Hereditary Leiomyomatosis and RCC

W. Marston Linehan, MD, chief, Urologic Oncology Branch, National Cancer Institute, discusses gene mutations in papillary kidney cancer.

Hereditary papillary renal carcinoma (HPRC) is a hereditary kidney cancer that is studied to help better understand non-hereditary kidney cancer. The patients with HRPC who have a germline MET-gene mutation are highly likely to develop bilateral, multifocal, type 1 papillary kidney cancer.

The MET-gene is mutated in a significant number of these patients, explains Linehan. The gene is altered in over 80% of tumors from patients with type 1 papillary kidney cancer.

Dr. Linehan on Gene Mutations in Papillary Kidney Cancer

W. Marston Linehan, MD, chief, Urologic Oncology Branch, National Cancer Institute, discusses genetic conditions with predisposition for patients with kidney cancer.

The goal was to understand the genes that cause kidney cancer, with the idea of studying their pathways and then developing therapies to target those pathways, explains Linehan. Kidney cancer is caused by 16 different genes, which respond differently to clinical therapies.

Studying gene families has been beneficial in understanding therapies for patients with sporadic non-inherited kidney cancer, states Linehan.

W. Marston Linehan, MD, chief, Urologic Oncology Branch, National Cancer Institute, discusses genetic conditions with predisposition for patients with kidney cancer. 

W. Marston Linehan, MD

Articles

Dr. Linehan on Treatment for Hereditary Leiomyomatosis and RCC

Dr. Linehan on Gene Mutations in Papillary Kidney Cancer

Dr. Linehan on Genes With Predisposition to Kidney Cancer