Management of hemangioblastomas in patients with von Hippel-Lindau disease: stereotactic radiosurgery compared to surgical excision

Symptom-producing HBs need treatment. Essentially all sporadic HBs are diagnosed as a result of the symptoms that they produce. A series of studies have shown that in the hands of experienced surgeons HBs of the cerebellum and brainstem can almost always be safely and completely removed surgically with a risk of recurrence approaching zero.2,5 Most symptom-producing HBs have either an associated cyst or a region of edema surrounding them, features associated with increased risk of adverse effects and poor tumor control after SRS, as is yet again demonstrated in the study by Kano and colleagues. The authors describe an incidence of 43% for new lesions in VHL patients at 5 years and 84% at 10 years. Among the lesions selected for treatment with SRS were new lesions.3 Do 84% of patients with VHL need to have SRS every 10 years? In patients with VHL, most HBs treated with radiosurgery have been small and asymptomatic. The incidence of progression of even small HBs treated with radiosurgery is not greatly different from their natural history if untreated. In VHL, since we cannot reliably predict which lesions will produce symptoms and require treatment, current knowledge favors introducing treatment when HBs produce symptoms and the use of surgery when that occurs. The bottom line for a patient with an HB producing symptoms? Find a neurosurgeon with significant surgical experience with these tumors. © AANS, 2015.