Nichols on Navigating the Nuances of von Hippel-Lindau Disease

Welcome to OncLive^® On Air! I’m your host today, [Gina Mauro].

OncLive On Air is a podcast from OncLive, which provides oncology professionals with the resources and information they need to provide the best patient care. In both digital and print formats, OncLive covers every angle of oncology practice, from new technology to treatment advances to important regulatory decisions.

Today, we had the pleasure of speaking with Kim E. Nichols, MD, of St. Jude Children’s Research Hospital, to discuss the incidence, prognosis, treatment, and ongoing research efforts in von Hippel-Lindau disease.

VHL disease is defined as a rare, inherited disorder that causes tumors and cysts to grow in specific areas of the body, including the brain, spinal cord, eyes, inner ear, adrenal glands, pancreas, kidney, and reproductive tract. Although many of these cases are benign, others can develop malignant tumors. Patients with VHL are also at an increased risk of presenting with rare types of tumors, including hemangioblastoma and pheochromocytomas.

However, those with VHL syndrome have an increased risk of certain types of other cancers, such as clear cell renal cell carcinoma (RCC) and neuroendocrine tumors of the pancreas. Approximately 70% of patients with VHL end up developing RCC by the age of 60 years and it is the leading cause of death.

In our exclusive interview, Dr. Nichols discussed VHL in great detail, how she currently approaches treatment with these patients, and the importance of routine surveillance in this population.