Cervicomedullary junction intramedullary hemangioblastoma

Abstract

Hemangioblastoma is a rare, benign, highly vascularized tumour, that usually presents a muriform nodule. The most frequent localization is the posterior cranial fossa. The frequency of this spinal lesion is very low, representing less than 5% of spinal cord tumours. The presentation of hemangioblastoma can widely vary, from a solid tumour to a lesion with a cystic component. We present the case of a 43 years old patient, admitted to our clinic for left hemiparesis, swallowing disorders, and dysphonia. An MRI is performed and it showed a cervicomedullary junction tumour with a solid nodule and an anterior cystic cavity pushing towards the 4th ventricle. The patient underwent surgery for the total removal of the tumour. Post-operative CT confirmed the total ablation of the tumour. The patient is discharged with improved symptomatology. In the last 10 years, in our clinic were admitted 23 cases of hemangioblastoma, 14 males (61% of the patients) and 9 females (39%) with an age at presentation varied from 14 to 78 years (mean 48,2 years). Only 3 patients (13%) out of the 23 had associated von Hippel–Lindau syndrome, with hemangioblastomas also present in other locations. The most common location was the posterior fossa, in 13 cases (56,6%). Headache (69,5%), ataxia (56%) and balance disorders (52%) were the most common symptoms accused by patients. The follow-up varied from 6 to 84 months (mean 20 months). Only one patient died during hospitalization and one tumour recurrence was noted. Most patients improved or remained clinically stable postoperatively.