Clinical features and long-term surgical outcomes of conus medullaris hemangioblastomas

圆锥血管母细胞瘤的临床特征和长期手术结果

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Abstract

BACKGROUND: Conus medullaris hemangioblastomas (HBs) are extremely rare vascular pathologies. The clinical-radiological features and long-term surgical outcomes of this rare disease are still lacking because of its rarity. OBJECTIVE: This single-center study was carried out to describe the clinical and radiological characteristics and surgical outcomes of this rare entity. METHODS: A cohort of patients who underwent resection of conus medullaris HBs in our center between 2009 and 2020 was retrospectively identified. Data on demographic features and surgical outcomes were collected and analyzed. RESULTS: Fourteen patients were consecutively included in this study (mean onset age of 43.2 ± 14.6 years, 57.1% male). Ten (71.4%) of them were in the sporadic group, whereas four (28.6%) had von Hippel-Lindau (VHL) syndrome. Gross total resection (GTR) was achieved in most patients (n = 13, 92.9%), whereas subtotal resection (STR) was achieved in the remaining patient (7.1%). During the 96.9 ± 41.9 (range: 39-172) months of follow-up, six patients (42.8%) showed improved function, four patients (28.6%) remained the same, two patients (14.3%) worsened, and two patients (14.3%) died. No patients experienced lesion recurrence. CONCLUSION: Conus medullaris HBs are rare spinal vascular entities. Timely and careful microsurgical dissection of HBs can provide symptomatic relief and halt the progression of neurological deficits. The surgical outcome is usually satisfactory, even in recurrent cases. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s41984-026-00548-4.

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