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Steven A. Mills, Michael C. Oh, Martin J. Rutkowski, Michael E. Sughrue, Igor J. Barani, Andrew T. Parsa, Supratentorial hemangioblastoma: clinical features, prognosis, and predictive value of location for von Hippel–Lindau disease, Neuro-Oncology, Volume 14, Issue 8, August 2012, Pages 1097–1104, https://doi.org/10.1093/neuonc/nos133
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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 Hippel–Lindau disease is positively correlated with supratentorial hemangioblastoma when compared with non-supratentorial CNS hemangioblastomas, particularly when present in the sellar/suprasellar region.