Early microsurgical treatment for spinal hemangioblastomas improves outcome in patients with von Hippel-Lindau disease.

Surgical Neurology International
Ali HaratiJuha Hernesniemi

Abstract

Spinal hemangioblastomas (HB) are rare, histologically benign, highly vascularized tumors often associated with von Hippel-Lindau (VHL) disease. The aim of the current study is to demonstrate the benefit of early surgical resection of large spinal HBs in selected asymptomatic patients with VHL. Seventeen patients underwent microsurgical resection of 20 spinal HBs at the Department of Neurosurgery at Helsinki University Central Hospital (HUCH). Thirteen tumors were in the cervical spine, five in thoracic and one patient had two lumbar lesions. MRI tumor showed an associated syrinx in 16 patients (94%). Tumor volume ranged from 27 to 2730 mm(3). Out of 17 patients, 11 (65%) tested positive for VHL in mutation analysis. Five of these patients with tumors ranging from 55 to 720 mm(3) were treated prophylactically. Complete tumor resection was performed in 16 patients (94%) who were followed up for a median of 57 months (range 2-165 months). No patient had neurological decline on long-term follow-up. Among the patients with VHL, five patients with preoperative sensorimotor deficits showed improvement of their symptoms but never regained full function. One patient who presented with tetraplegia remained the same. Otherwise, all five ...Continue Reading

Methods Mentioned

BETA
dissection
prophylactic surgery
biopsy
prophylactic resection

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