Takeshima Yasuhiro, Takami Hirokazu, Endo Toshiki, Mizuno Masaki, Hida Kazutoshi
Department of Neurosurgery, Nara Medical University, Kashihara, Japan.
Department of Neurosurgery, Faculty of Medicine, The University of Tokyo Hospital, Tokyo, Japan.
Neurospine. 2023 Sep;20(3):756-765. doi: 10.14245/ns.2346368.184. Epub 2023 Jun 20.
This study aimed to clarify the relationship between recurrence and the extent of resection in surgery for intramedullary spinal hemangioblastoma (sHB) and its impact on von Hippel-Lindau (vHL) disease.
Data on sHB cases followed up for at least 6 months after surgery were extracted from a nationwide registry of 1,033 consecutive spinal intramedullary tumors surgically treated between 2009 and 2020, and were retrospectively categorized into a sporadic or vHL group. The diagnosis of vHL disease was made at each institution based on clinical findings.
A total of 168 patients (sporadic group, 101; vHL group, 67) were included in the study. Compared with the sporadic group, the vHL group had a younger onset (45.4 ± 16.8 years vs. 39.6 ± 14.1 years, p = 0.02), more preoperative motor (47.5% vs. 68.7%, p < 0.01) and gait (37.6% vs. 61.2%, p < 0.01) impairments, and more patients with worsening neurological symptoms at discharge (p = 0.02). The gross total resection (GTR) rates and the recurrence rates were not statistically different between the sporadic and the vHL groups. GTR significantly improved recurrence-free survival compared to non-GTR in all patient analysis (p < 0.01) but this trend was not observed in the sporadic group. Physical functional improvement from discharge to 6 months after surgery was observed in the sporadic group (p < 0.01) but not in the vHL group.
A high GTR rate may sufficiently decrease susceptibility to recurrence, especially in patients with sHB with vHL. In sporadic sHB, postoperative functional improvement can be expected, and the long-term functional prognosis is favorable.
本研究旨在阐明髓内脊髓血管母细胞瘤（sHB）手术切除范围与复发之间的关系及其对冯·希佩尔-林道（vHL）病的影响。
从2009年至2020年期间接受手术治疗的1033例连续性脊髓髓内肿瘤的全国性登记中提取术后至少随访6个月的sHB病例数据，并回顾性地分为散发性或vHL组。vHL病的诊断由各机构根据临床表现做出。
本研究共纳入168例患者（散发性组101例；vHL组67例）。与散发性组相比，vHL组发病年龄更小（45.4±16.8岁对39.6±14.1岁，p = 0.02），术前运动功能障碍（47.5%对68.7%，p < 0.01）和步态障碍（37.6%对61.2%，p < 0.01）更多，出院时神经症状恶化的患者更多（p = 0.02）。散发性组和vHL组的全切除（GTR）率和复发率无统计学差异。在所有患者分析中，与非GTR相比，GTR显著改善了无复发生存率（p < 0.01），但在散发性组中未观察到这种趋势。散发性组术后从出院到术后6个月观察到身体功能改善（p < 0.01），而vHL组未观察到。
高GTR率可能足以降低复发易感性，尤其是在患有vHL的sHB患者中。在散发性sHB中，术后功能改善是可以预期的，长期功能预后良好。
