Dr. Nichols on the Prognosis of Patients With VHL Disease

Kim E. Nichols, MD, member, St. Jude Faculty, director, Cancer Predisposition Division, Oncology Department, St. Jude Children’s Research Hospital, discusses the prognosis of patients with von Hippel-Lindau (VHL) disease.

Patients with VHL disease, particularly pediatric patients, have favorable prognoses, says Nichols. With the exceptions of renal cell carcinoma, neuroendocrine tumors, and adrenal tumors, the common tumors that arise in patients with VHL disease are benign, Nichols explains.

Although these tumors don’t necessarily increase mortality by metastasizing, they can grow slowly and impede vital organs they are embedded in or surrounding. For example, endolymphatic sac tumors are small, locally invasive tumors that can occur in the inner ear. When these tumors push on the auditory nerves, they can lead to deafness.

Although patients with VHL disease typically have a normal life span, they require close monitoring ensure tumors do not lead to organ dysfunction, concludes Nichols.