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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 Neurospine Society of Japan

Neurospine. 2023 Jun 20. doi: 10.14245/ns.2346368.184. Online ahead of print.

ABSTRACT

OBJECTIVE: 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.

METHODS: 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.

RESULTS: 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 v.s. 39.6±14.1 years, p=0.02), more preoperative motor (47.5 v.s. 68.7%, p<0.01) and gait (37.6 v.s. 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.

CONCLUSION: 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.

PMID:37350167 | DOI:10.14245/ns.2346368.184

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