Glioneuronal Heterotopia Presenting as Cerebellopontine Angle Tumor of Cranial Nerve VIII

BACKGROUND: Vestibular schwannomas and meningiomas account for the great majority of lesions arising in the cerebellopontine angle. In this report, we present a case of glioneuronal heterotopia, also known as glioneuronal hamartoma, arising from cranial nerve VIII, which is an extremely uncommon lesion. Important radiologic and surgical aspects are reviewed, which may help in early recognition and intraoperative decision making when these lesions are encountered. CASE DESCRIPTION: A healthy 29-year-old female presented with intermittent right facial numbness. Magnetic resonance imaging showed an incidental, minimally enhancing cerebellopontine angle lesion on the right cranial nerve VII-VIII complex. The patient declined serial observation and opted for operative intervention for resection. Intraoperatively, the lesion resembled neural tissue and was continuous with the VIII cranial nerve. Pathologic analysis demonstrated mature glioneuronal tissue consistent with hamartomatous brain tissue. The patient maintained normal hearing and facial nerve function after surgery. Radiologic, surgical, and pathologic characteristics are described. CONCLUSIONS: Ectopic glioneuronal tissue of cranial nerve VIII is a rare non-neoplastic lesion and should be considered in the differential diagnosis of unusual-appearing intracanalicular and cerebellopontine angle lesions. The congenital and benign nature of this entity makes observation a valid option for these cases, although they are so infrequent that they are often presumptively managed as vestibular schwannomas. Attempts to radically resect these lesions may result in higher rates of hearing loss or facial palsy due to their continuity with cranial nerves.