TREATMENT OF HEMANGIOBLASTOMAS IN VON-HIPPEL-LINDAU-DISEASE WITH LINEAR ACCELERATOR-BASED RADIOSURGERY

OBJECTIVE: Stereotactic radiosurgery is increasingly being used to tre at hemangioblastomas, particularly those that are in surgically inacce ssible locations or that are multiple, as is common in von Hippel-Lind au disease. The purpose of this study was to retrospectively evaluate the effectiveness of radiosurgery in the treatment of hemangioblastoma s. METHODS: From 1989 to 1996, 29 hemangioblastomas in 13 patients wit h von Hippel-Lindau disease were treated with linear accelerator-based radiosurgery. The mean patient age was 40 years (range, 31-57 yr). Th e radiation dose to the tumor periphery averaged 23.2 Gy (range, 18-40 Gy).The mean tumor volume was 1.6 cm(3) (range, 0.07-65.4 cm(3)). Tum or response was evaluated in serial, contrast-enhanced, computed tomog raphic and magnetic resonance imaging scans. The mean follow-up period was 43 months (range, 11-84 mo). RESULTS: Only one (3%) of the treate d hemangioblastomas progressed. Five tumors (17%) disappeared, 16 (55% ) regressed, and 7 (24%) remained unchanged in size. Five of nine pati ents with symptoms referable to treated hemangioblastomas experienced symptomatic improvement. During the follow-up period, one patient died as a result of progression of untreated hemangioblastomas in the cerv ical spine. Three patients developed radiation necrosis, two of whom w ere symptomatic. CONCLUSION: Although follow-up monitoring is limited, stereotactic radiosurgery provides a high likelihood of local control of hemangioblastomas and is an attractive alternative to multiple sur gical procedures for patients with von Hippel-Lindau disease.