ScientiaCME Challenges and updates in the management of von Hippel-Lindau disease and related tumors
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Activity Description / Statement of Need:
In this online, self-learning activity:
Von Hippel-Lindau (VHL) disease is a rare genetic condition caused by an autosomal dominant mutation of the VHL tumor suppressor gene. The mutation gives rise to an abnormal VHL protein that cannot bind effectively to protein HIF-1α, leading to the transcription of multiple genes and upregulation of growth factors. The condition is thought to affect between 1 in 39,000 and 1 in 91,000, with a birth incidence of between 1 in 36,000 and 1 in 45,500. Diagnosis of VHL disease is established when the patient undergoes genetic testing and a pathogenic mutation of the VHL gene is found. Genetic testing is typically conducted if the patient has a family history of VHL or they are showing signs of VHL-related symptoms. VHL disease is characterized by tumors and cysts growing in various parts of the body, including the brain, spine, eyes, inner ears, pancreas, kidneys, adrenal glands, and reproductive tract. The clinical presentation of the disease is different in every patient and is impossible to predict, so close monitoring is required. Complications of the disease are on a case-by-case basis, but patients with VHL are at an increased risk of developing some cancers, particularly clear cell renal cell carcinoma (RCC) and pancreatic cancer.
HCPs including: Medical and genitourinary oncologists, urologists, and nephrologists; physician assistants, nurse practitioners, and pharmacists specializing in the aforementioned areas of specialty; and any other HCPs involved or interested in treatment of VHL disease.