Prospective evaluation of radiosurgery for hemangioblastomas in von Hippel-Lindau disease
Open Access
- 23 December 2009
- journal article
- clinical trial
- Published by Oxford University Press (OUP) in Neuro-Oncology
- Vol. 12 (1), 80-86
- https://doi.org/10.1093/neuonc/nop018
Abstract
To determine the effectiveness of stereotactic radiosurgery (SRS) treatment to central nervous system (CNS) hemangioblastomas in von Hippel–Lindau disease (VHL), we analyzed long-term results in VHL patients treated with SRS. Patients were enrolled in a prospective VHL natural history study, undergoing SRS treatment of CNS hemangioblastomas. Treatment regimens, serial clinical evaluations, and longitudinal imaging data were analyzed. Twenty VHL patients (10 males and 10 females) underwent SRS treatment of 44 CNS hemangioblastomas (39 cerebellar and 5 brainstem). Mean (±SD) age at treatment was 37.5 ± 12.0 years (range: 13–67). Mean follow-up was 8.5 ± 3.2 years (range: 3.0–17.6 years). All patients were alive at last follow-up. Mean treated tumor volume was 0.5 ± 0.7 cm3 (range: 0.01–3.6 cm3). Mean prescription dose was 18.9 Gy (range: 12–24 Gy) at the tumor margin. Local control rate at 2, 5, 10, and 15 years after SRS treatment was 91%, 83%, 61%, and 51%, respectively. Univariate analysis did not identify variables associated (P > .05) with worse tumor control at last follow-up. Thirty-three percent of SRS-treated small (<1.0 cm diameter), asymptomatic tumors progressed over a long-term follow-up. There were no long-term adverse radiation effects. Although SRS treatment of hemangioblastomas in VHL has a low risk for adverse radiation effects, it is associated with diminishing control over a long-term follow-up. These results indicate that SRS should not be used to prophylactically treat asymptomatic tumors and should be reserved for the treatment of tumors that are not surgically resectable.Keywords
This publication has 30 references indexed in Scilit:
- Neurologic Manifestations of von Hippel-Lindau DiseaseJAMA, 2008
- Gamma knife radiosurgery for intracranial haemangioblastomasActa Neurochirurgica, 2007
- Edema is a precursor to central nervous system peritumoral cyst formationAnnals of Neurology, 2005
- The long-term results of gamma knife radiosurgery for hemangioblastomas of the brainJournal of Neurosurgery, 2005
- Gamma knife surgery for multiple hemangioblastomasJournal of Neurosurgery, 2005
- Stereotaxic radiosurgical ablation: An alternative treatment for recurrent and multifocal hemangioblastomas: A report of four casesSurgical Neurology, 1993
- Identification of the von Hippel-Lindau Disease Tumor Suppressor GeneScience, 1993
- Volume of Pituitary MacroadenomasJournal of Computer Assisted Tomography, 1992
- Von Hippel–Lindau disease maps to the region of chromosome 3 associated with renal cell carcinomaNature, 1988
- The use of the nitrogen mustards in the palliative treatment of carcinoma.With particular reference to bronchogenic carcinomaCancer, 1948