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

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 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.

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.

Keywords: Hemangioblastoma; Intramedullary spinal cord neoplasms; Recurrence; Spinal cord; Treatment outcome; Von Hippel-Lindau disease.

Fig. 1.

Kaplan-Meier plot demonstrating recurrence-free survival (RFS) after resection of intramedullary hemangioblastoma. Stratified analysis by the presence of von Hippel-Lindau (vHL) disease (A) and extent of resection (B) in all patients. No significant difference is observed between the 2 groups in terms of RFS (p=0.27). In contrast, gross total resection (GTR) improved RFS (p<0.01). Stratification according to the extent of resection in the sporadic (C) and vHL groups (D). As opposed to the sporadic group, non-GTR tended to shorten the RFS in the vHL group. Patients at risk are indicated.

Fig. 2.

Perioperative changes in the functional status in the sporadic (A) and von Hippel-Lindau (vHL) group (B). There are significant differences in the distribution of the modified McCormick scale between discharge and 6 months postoperatively (p<0.01), and between the preoperative period and final follow-up (p<0.01) in the sporadic group. However, there is no significant difference in any assessment interval in the vHL group. NS, not significant. Grade I, normal gait; grade II, mild gait disturbance not requiring support; grade III, gait with support; grade IV, assistance required; and grade V, wheelchair needed. *p<0.05.