The aim was to identify predictors of progression in a series of patients managed for an intracranial hemangioblastoma, in order to guide the postoperative follow-up modalities. The characteristics of 81 patients managed for an intracranial hemangioblastoma between January 2000 and October 2022 were retrospectively analyzed. The mean age at diagnosis was of 48 ± 16 years. Eleven (14%) patients had von Hippel–Lindau disease. The most frequent tumor location was the cerebellar hemispheres (n= 51, 65%) and 11 (14%) patients had multicentric hemangioblastomas. A gross total resection was achieved in 75 (93%) patients. Eighteen (22%) patients had a local progression, with a median progression-free survival of 56 months 95% CI [1;240]. Eleven (14%) patients had a distant progression (new hemangioblastoma and/or growth of an already known hemangioblastoma). Local progression was more frequent in younger patients (39 ± 14 years vs. 51 ± 16 years;p= 0.005), and those with von Hippel–Lindau disease (n= 8, 44% vs.n= 3, 5%,p< 0.0001), multiple cerebral locations (n= 3, 17% vs.n= 2, 3%,p= 0.02), and partial tumoral resection (n= 4, 18% vs.n= 1, 2%,p= 0.0006). Therefore, it is advisable to propose a postoperative follow-up for at least 10 years, and longer if at least one predictor of progression is present.
本研究旨在识别颅内血管母细胞瘤患者疾病进展的预测因素，以指导术后随访策略。回顾性分析了2000年1月至2022年10月期间收治的81例颅内血管母细胞瘤患者的临床特征。确诊时平均年龄为48±16岁，其中11例（14%）患者患有希佩尔-林道综合征。肿瘤最常见于小脑半球（51例，65%），11例（14%）患者存在多发性血管母细胞瘤。75例（93%）患者实现了肿瘤全切除。18例（22%）患者出现局部进展，中位无进展生存期为56个月（95% CI [1;240]）。11例（14%）患者发生远处进展（新发血管母细胞瘤和/或已知病灶增大）。局部进展更常见于年轻患者（39±14岁 vs. 51±16岁；p=0.005）、希佩尔-林道综合征患者（8例[44%] vs. 3例[5%]；p<0.0001）、多发性颅内病灶患者（3例[17%] vs. 2例[3%]；p=0.02）以及肿瘤部分切除患者（4例[18%] vs. 1例[2%]；p=0.0006）。因此建议术后至少随访10年，若存在任一进展预测因素则应延长随访时间。
肿瘤(癌症)患者之家