Comparison of the Recurrence and Surgical Outcome of Spinal Hemangioblastoma in Sporadic and Von Hippel-Lindau Diseases: A Subanalysis of a Nationwide Study by the Neurospinal Society of Japan.

objectiveThis 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.

methodsData 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.

resultsA 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.

conclusionA 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.