Intramedullary hemangioblastomas: timing of surgery, microsurgical technique and follow-up in 23 patients

Abstract

Hemangioblastomas are highly vascularised tumors of the central nervous system and account for 1.5–2.5% of all spinal cord tumors. Because of the rarity of these tumors, surgical experience is often limited and, therefore, treatment and indications for timing of surgery are discussed controversial. The authors reviewed their data of 23 consecutive patients with respect to timing of surgery, microsurgical technique, and follow-up. Clinical records of 23 consecutive patients with intramedullary hemangioblastomas who underwent first surgery in our department between 1990 and 2005 were reviewed. In three cases the tumors were localised at the craniocervical junction; four patients had a single tumor in the cervical spine, six patients multiple tumors in the cervical and thoracic spine, eight patients in the thoracic spine only, one patient in the conus region, and one patient had multiple tumors located in the thoracic and lumbar spine. In eight patients, a von-Hippel-Lindau disease (VHL) was associated. The neurological follow-up was evaluated according to the classification of McCormick. Operation was recommended to every symptomatic patient as early as possible. In asymptomatic patients with a sporadic tumor surgery was discussed for diagnostic purposes at any time. In VHL patients, surgery was recommended if tumor growth was observed on MRI in the next practicable time. All tumors were diagnosed by magnetic resonance imaging and in all cases but one a DSA was performed. All patients were treated microsurgically through a posterior approach. The tumors in the spinal cord were removed microsurgically through a partial hemilaminectomy (n = 1), a hemilaminectomy (n = 15), or laminectomy (n = 4) and at the craniocervical junction (n = 3) through a suboccipital craniotomy. During follow-up after 6 months, 18 patients remained neurologically stable (17 in McCormick grade I and 1 in McCormick grade II) and 5 patients recovered to a better status (3 from grade III to II, 2 from grade II to I). There was one complication with a CSF fistula and one recurrence/incomplete removal. Following the above-mentioned principles of microsurgical removal of intramedullary hemangioblastomas, operation is possible with a low procedure-related morbidity and can be recommended especially in VHL patients with progressive symptoms or tumor growth during follow-up. Patients without VHL most frequently require hemangioblastoma resection for diagnostic purposes and/or because symptoms prompted an imaging work-up that lead to the discovery of the tumor.