Clinical and surgical features of lower brain stem hemangioblastomas in von Hippel-Lindau disease.

BACKGROUND: In the context of von Hippel-Lindau disease (VHL), the medulla oblongata is a relatively frequent site of growth of hemangioblastomas, posing related clinical and surgical difficulties. Their management requires a close correlation between clinical evolution and morphological surveillance. In order to describe their clinical and surgical features, we reviewed our experience in the treatment of these lesions.

METHODS: Between 2001 and 2009, 14 patients (9 female and 5 male, mean age 34 years) underwent removal of 15 lower brain stem hemangioblastomas. Based on the review of the clinical records and outpatient long-term follow-up visits, their clinical course was analyzed. Functional evaluation was measured with the Karnofsky Performance Scale (KPS) on admission, at discharge and at the last follow-up. The mean follow-up period was 30.8 months (range 4-99).

RESULTS: All the operated hemangioblastomas were located in the dorsal medulla oblongata, in the context of multiple lesions, cerebellar and/or spinal. In ten patients hemangioblastomas were located in a median position at the obex area; in four cases a lateral location was observed. Cystic component was absent in two cases. Clinical onset preceded surgery by a mean of 8.5 months. Preoperatively three patients showed a KPS lower than 80, ten patients between 80 and 90, and one patient scored 100 (asymptomatic). There was no surgical mortality. Nine out of 14 patients showed a temporary surgical morbidity. One patient required a tracheostomy. At follow-up ten patients scored a KPS better than before the operation, while the other four patients remained stable. Permanent morbidity was observed in three patients.

CONCLUSIONS: Lower brain stem hemangioblastomas in the context of VHL show an often gradual onset of signs and symptoms except for patients who develop an obstructive hydrocephalus. Although transient surgical complications are possible, surgery provides favorable long-term results.