Multifocal cerebral hemangio-endothelioma. Case report and review of the literature

Background. Intracranial hemangio-endotheliomas (HE) are rare vascular tumo urs that present histological features and biological behaviour of an inter mediate malignancy. We report the first case of primary HE presenting as mu ltiple intracranial masses. Case Description. The patient is a 20-year-old female who presented with pa resis of the left arm. Magnetic Resonance imaging (MRI) showed multiple les ions in both frontal lobes. Total surgical excision of the tumours was obta ined: a histological diagnosis of hemangio-endothelioma was made. Five mont hs later, MRI scan showed new intradiploic skull lesions whereas a scintigr aphic study revealed multicentric extracranial bone involvement. Treatment based on interferon-alpha (IFN-alpha) administration was undertaken; this p roduced partial regression of the lesions. Approximately three years after the first operation, the patient is in good clinical condition. Conclusions. Surgery continues to play the leading role in the treatment of intracranial HE. However, adjuvant therapy with IFN represents a new and w ell-tolerated therapeutic method for residual intracranial lesions as well as multicentric extracranial disease.