Treatment of hemangioblastomas in von Hippel-Lindau disease with linear accelerator-based radiosurgery

Neurosurgery. 1998 Jul;43(1):28-34; discussion 34-5. doi: 10.1097/00006123-199807000-00018.


Objective: Stereotactic radiosurgery is increasingly being used to treat hemangioblastomas, particularly those that are in surgically inaccessible locations or that are multiple, as is common in von Hippel-Lindau disease. The purpose of this study was to retrospectively evaluate the effectiveness of radiosurgery in the treatment of hemangioblastomas.

Methods: From 1989 to 1996, 29 hemangioblastomas in 13 patients with von Hippel-Lindau disease were treated with linear accelerator-based radiosurgery. The mean patient age was 40 years (range, 31-57 yr). The radiation dose to the tumor periphery averaged 23.2 Gy (range, 18-40 Gy). The mean tumor volume was 1.6 cm3 (range, 0.07-65.4 cm3). Tumor response was evaluated in serial, contrast-enhanced, computed tomographic and magnetic resonance imaging scans. The mean follow-up period was 43 months (range, 11-84 mo).

Results: Only one (3%) of the treated hemangioblastomas progressed. Five tumors (17%) disappeared, 16 (55%) regressed, and 7 (24%) remained unchanged in size. Five of nine patients 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 cervical spine. Three patients developed radiation necrosis, two of whom were 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 surgical procedures for patients with von Hippel-Lindau disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Brain Neoplasms / diagnosis
  • Brain Neoplasms / mortality
  • Brain Neoplasms / surgery*
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Hemangioblastoma / diagnosis
  • Hemangioblastoma / mortality
  • Hemangioblastoma / surgery*
  • Humans
  • Male
  • Middle Aged
  • Radiosurgery*
  • Retrospective Studies
  • Spinal Cord Neoplasms / diagnosis
  • Spinal Cord Neoplasms / mortality
  • Spinal Cord Neoplasms / surgery*
  • Survival Rate
  • Treatment Outcome
  • von Hippel-Lindau Disease / diagnosis
  • von Hippel-Lindau Disease / mortality
  • von Hippel-Lindau Disease / surgery*