TY - JOUR
T1 - Supratentorial hemangioblastoma
T2 - Clinical features, prognosis, and predictive value of location for von HippelLindau disease
AU - Mills, Steven A.
AU - Oh, Michael C.
AU - Rutkowski, Martin J.
AU - Sughrue, Michael E.
AU - Barani, Igor J.
AU - Parsa, Andrew T.
PY - 2012/8
Y1 - 2012/8
N2 - 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.
AB - 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.
KW - hemangioblastoma
KW - supratentorial
KW - von Hippel-Lindau
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U2 - 10.1093/neuonc/nos133
DO - 10.1093/neuonc/nos133
M3 - Review article
C2 - 22723428
AN - SCOPUS:84864719743
SN - 1522-8517
VL - 14
SP - 1097
EP - 1104
JO - Neuro-Oncology
JF - Neuro-Oncology
IS - 8
ER -