Stereotactic radiosurgery for central nervous system hemangioblastoma in von Hippel-Lindau disease: A systematic review and meta-analysis

Abstract

Von Hippel-Lindau (VHL) disease is a dominantly inherited disorder marked by multiorgan tumors, such as central nervous system benign hemangioblastomas (CHB). Stereotactic radiosurgery (SRS) has also been used to treat CHB for a long time. The purpose of this meta-analysis is to provide a long-term outcome of SRS for VHL-associated CHB by reviewing published studies. We completed a Pubmed/Embase/SCOPUS/Cochrane Library literature search to get eligible studies published from January 1990 to December 2019 about using SRS to treat VHL-associated CHB. 15 studies met eligibility for qualitative systematic review, of which nine studies were ultimately eligible for quantity meta-analysis of 5-year tumor control rates (TCR), representing 170 subjects with a total of 660 lesions. Gamma Knife was the most published SRS method for VHL-associated CHB. The pooled 5-year TCR across the nine studies was 0.919 (95 %CI: 0.881−0.957). The pooled 5-year TCR for only intracranial lesions across eight studies was 0.917 (95 %CI: 0.876−0.957). Individual patient data were extracted from 9 studies, representing 298 lesions of 70 subjects. Sex, tumor volume, radiosurgery methods, marginal doses, maximum doses, the number of tumors for radiosurgery, age at the time of radiosurgery, tumor locations were not proven to be associated with tumor progression. SRS offered a satisfactory 5-year tumor control of CHB for VHL patients. Despite the paucity of randomized control trials, SRS is recommended to patients with limited surgical alternatives. However, the long-term outcomes and underlying factors associated with tumor progression remain to be investigated.