[Intraspinal hemangioblastoma. Apropos of a recent series of 20 cases]

Abstract

This report deals with a series of 20 patients admitted for a spinal hemangioblastoma between 1970 and 1993, 18 of whom between 1978 and 1993. There are 8 men and 12 women and the onset of spinal symptoms is before the age of 50 years for 76% of the patients. Nineteen patients have an intra-dural tumor (extramedullary 15.5%, intramedullary 75% with 62.5% close to the dorsal surface, 37.5% partly out, none totally enclosed with spinal cord) and only one patient has an extra-dural tumor. Cysts are present in 70% of our cases. Two cases have been revealed by an hemorrhagic syndrome. Ten cases belong to a von Hippel-Lindau (vHL) disease. Nineteen patients were operated on, one patient died. Total resection of the tumor was performed in 17. Symptoms improved postoperatively in 8 patients, were inchanged in 6, and worsened temporary in 4. MRI with gadolinium is the best procedure for both the diagnosis and the follow-up. After surgery, the prognosis is very good for sporadic cases with a single spinal lesion whereas it is very poor in case of vHl disease. For these patients (50% of our cases), half of them died because of the evolution of multifocal lesions even if they were operated on several times. Thus, each patient should have a precise check-up (SNC and visceral lesions). The gene causing vHL disease located on the short arm of the chromosome 3 has just been characterized. This will help to elaborate a presymptomatic diagnosis and a better screening of the patients with the hope of a specific treatment.