Supratentorial hemangioblastoma: Clinical features, prognosis, and predictive value of location for von HippelLindau disease

Steven A. Mills, Michael C. Oh, Martin J. Rutkowski, Michael E. Sughrue, Igor J. Barani, Andrew T. Parsa

Research output: Contribution to journalReview article

20 Scopus citations

Abstract

Supratentorial hemangioblastoma is a rare form of hemangioblastoma; little information is available regarding prognosis, treatment, and clinical characteristics, because the available literature is primarily composed of case reports and small case series. Therefore, we performed a systematic review of the literature to analyze clinical characteristics, disease progression, and surgical outcomes with respect to survival for supratentorial hemangioblastomas. The rate of progression-free survival (PFS) was determined using Kaplan-Meier analysis. Differences in categorical factors, including location of tumor and diagnosis of von Hippel-Lindau (VHL) disease, were analyzed using the Pearson χ2 test. A total of 106 articles met the search criteria, which combined for a total of 132 patients. Of the patients with supratentorial tumors, 60 had VHL disease, and 31 (84) of 37 patients with tumors in the sellar/suprasellar region had associated VHL (χ2, P <. 001). Five-year PFS for gross-total resection and subtotal resection were 100 and 53, respectively (Log rank, P <. 01). On the basis of our analysis of the literature on published cases of supratentorial hemangioblastoma, gross-total resection appears to be superior to other treatment modalities in extending PFS. Von HippelLindau disease is positively correlated with supratentorial hemangioblastoma when compared with non-supratentorial CNS hemangioblastomas, particularly when present in the sellar/suprasellar region.

Original languageEnglish (US)
Pages (from-to)1097-1104
Number of pages8
JournalNeuro-Oncology
Volume14
Issue number8
DOIs
StatePublished - Aug 2012
Externally publishedYes

Keywords

  • hemangioblastoma
  • supratentorial
  • von Hippel-Lindau

ASJC Scopus subject areas

  • Oncology
  • Clinical Neurology
  • Cancer Research

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