Introduction: Hemangioblastomas (HBs) are benign, highly vascular tumors that can be found intracranially or in the spinal region, representing around 2–15% of primary intramedullary tumors. They can occur sporadically or in association with Von Hipple–Lindau (VHL) disease. Despite recent of advancement of nonsurgical treatments, complete surgical resection remains the gold standard of care for the spinal HBs. Materials and Methods: We conducted an international multicenter retrospective analysis of adult patients surgically treated for spinal HBs in four European referral centers between January 2000 and September 2024, with a minimum post-operative follow-up duration of 6 months. Patients’ sex and age at surgical intervention, clinical presentation, and duration symptoms prior to clinical diagnosis were identified. The pre- and post-operative neurological status at 1 and 6 months and at the last visit was assessed using the modified McCormick score (MCS). The extent of surgical resection was divided into gross total resection (GTR) and subtotal resection (STR). Finally, post-operative complications were inspected as well, namely cerebrospinal fluid leaks, infections, hemorrhages and post-operative spinal stability. Results: A total of 35 patients were included in the cohort, with an age median of 52 years (34.5–60) and a slight male predominance (21/35, i.e., 60%). The median follow-up period was 37.5 months (12–75). More than half were located in the cervical region, making it the most common (54.3%). Syrinxes were observed in 23 cases (72%), and HBs were more commonly intramedullary (80%). GTR was achievable in around 88% of cases. Post-operative complications were observed in nine patients (25.7%). Nearly half of patients were discharged into rehabilitations centers (48.5%). Tumor recurrence was seen in 10.3% only. At the last follow-up, an excellent overall post-operative neurological status (positive ∆ McCormick) was observed in most of patients (88%) and was found to be associated with a relatively younger age group. Tumor location and presence of syrinxes did not show any statistical significance regarding clinical outcome. In patients having benefited from intra-operative monitoring, only D-wave changes showed statistical significance regarding post-operative outcome (p< 0.05). Conclusions: A large majority of patients operated for a spinal HB demonstrated favorable outcome after surgery, with unchanged or improved neurological status. Advanced age could have an impact on the post-operative neurological outcome. Other factors such as tumor size, location, and the presence of syrinx did not seem to significantly impact the neurological outcome. Finally, the surgery of these vascular lesions with no possibility of debulking or piece-meal removal and requiring “en bloc” resection is technically demanding and should be performed by experienced teams in spine and spinal cord surgery only.
引言：血管母细胞瘤（HBs）是一种良性高血管性肿瘤，可发生于颅内或脊髓区域，约占原发性髓内肿瘤的2-15%。该肿瘤可散发发生，也可与Von Hipple-Lindau（VHL）病相关。尽管近年来非手术治疗有所进展，但完全手术切除仍是脊髓血管母细胞瘤治疗的金标准。 材料与方法：我们对2000年1月至2024年9月期间在欧洲四家转诊中心接受手术治疗的脊髓血管母细胞瘤成年患者进行了国际多中心回顾性分析，术后随访时间至少6个月。收集患者性别、手术时年龄、临床表现及临床诊断前症状持续时间等数据。采用改良McCormick评分（MCS）评估术前及术后1个月、6个月和末次随访时的神经功能状态。手术切除程度分为大体全切除（GTR）和次全切除（STR）。同时记录术后并发症，包括脑脊液漏、感染、出血及术后脊柱稳定性问题。 结果：共纳入35例患者，中位年龄52岁（34.5-60岁），男性略占优势（21/35，即60%）。中位随访期为37.5个月（12-75个月）。超过半数肿瘤位于颈段（54.3%），为最常见部位。23例（72%）患者合并脊髓空洞，80%的肿瘤为髓内型。约88%的病例可实现大体全切除。9例患者（25.7%）出现术后并发症。近半数患者（48.5%）出院后转入康复中心。肿瘤复发率仅为10.3%。末次随访时，绝大多数患者（88%）术后总体神经功能状态良好（McCormick评分改善），且与相对年轻的年龄组相关。肿瘤位置及是否合并脊髓空洞对临床结局无统计学影响。在术中监测获益的患者中，仅D波变化与术后结局具有统计学显著性（p<0.05）。 结论：绝大多数接受手术的脊髓血管母细胞瘤患者术后结局良好，神经功能状态保持稳定或得到改善。高龄可能对术后神经功能结局产生影响。肿瘤大小、位置及是否合并脊髓空洞等其他因素似乎对神经功能结局无显著影响。最后，此类血管性病变无法进行减瘤或分块切除，需行整块切除，手术技术要求高，仅应由经验丰富的脊柱脊髓外科团队实施。
肿瘤(癌症)患者之家