gms | German Medical Science

Objective: Hemangioblastomas are rare highly vascularized tumors of the central nervous system accounting for 1.5 - 2.5% of all spinal cord tumors. They occur in 20-30% of von Hippel-Lindau (VHL) disease and are often multiple in these cases. Because of the low incidence of these tumors, surgical experience is limited and, therefore,indications for the timing of surgery are controversial. The authors reviewed their data of 21 patients with respect to timing of surgery, microsurgical technique and follow-up.

Methods: Clinical records of 21 patients with intramedullary hemangioblastomas, who underwent first surgery in our department between 1990-2005, were reviewed. In 3 cases the tumors were localized in the medulla, 4 patients had a single tumor in the cervical spine (CS), 4 patients had multiple tumors in the cs and thoracic spine (ts), 8 in ts only, 1 in the conus-medullare region, 1 had multiple tumors located in the thoracic and lumbar spine. In 8 (38%) patients a VHL was associated. Follow-up was evaluated according to the classification of McCormick.

Results: All tumors were diagnosed by magnetic resonance imaging and in all cases but one a DSA was performed. All patients were treated by microsurgery through a posterior approach. Tumors were removed in the spinal cord through a partial hemilaminectomy, a hemilaminectomy or aa laminectomy,. INn the medulla oblongata (n=3)the tumors are approached through a suboccipital trepanation. The strategy of the microsurgical removal of the tumor was to coagulate the dominant arterial feeder first, then coagulate and shrink the tumor and, finally, coagulate the venous drainage. Only patients with new onset of or progressive neurological deficits or with tumor progress in the follow-ups were treated by surgery. Postoperatively, 15 patient remained neurologically stable, all were in McCormick grade I. 5 patients recovered to a better status: 4 from grade II to I and 1 from grade III to II. 1 patient showed a temporary worsening of his symptoms but recovered to a status that was better than the pre-operative status during follow-up. Only 1 complication with a CSF-fistula and 1 recurrence/incomplete removal.1 patient underwent surgery twice, as the tumor couldn`t be identified during the first surgery.

Conclusions: Following the above mentioned principles of microsurgical removal of spinal hemangioblastomas, surgery is possible with a low procedure-related morbidity and can be recommended in patients with progressive symptoms or tumor growth during follow-up.