Extraaxial primitive neuroectodermal tumor mimicking a vestibular schwannoma: diagnostic and therapeutic difficulties. Report of two cases.

摘要

Extraaxial cerebellopontine angle (CPA) medulloblastomas and other primitive neuroectodermal tumors (PNETs) are rare tumors. The authors report on two patients with PNETs who presented with progressive audiovestibular symptoms. In each case magnetic resonance (MR) imaging revealed an extraaxial lesion that filled the internal auditory meatus and exhibited the neuroimaging features of a vestibular schwannoma (VS). No high signal intensity was apparent in either the brainstem or adjacent cerebellum on T2-weighted MR images. Surgery with maximum resection (total in one case and subtotal in the other) was performed, followed by craniospinal radiotherapy. One year postoperatively, both patients were free from tumor. A CPA PNET mimicking a VS is a rare entity, the diagnosis of which is important because its treatment differs dramatically from that of VS, including prescribed surgery followed by conventional craniospinal radiotherapy.