Primary malignant melanoma of the cerebellopontine angle.

BACKGROUND: Malignant tumors of the cerebellopontine angle are very rare, accounting for less than 1% of lesions at this site. These may be primary or secondary tumors of the temporal bone, central nervous system (CNS), or leptomeninges. Malignant melanoma is uncommon, accounting for 1.5% of all types of malignant tumors. Metastatic melanoma is a frequent cause of CNS metastasis, often with leptomeningeal spread. Primary leptomeningeal melanoma is, however, rare and even more so at the cerebellopontine angle. The prognosis for CNS malignant melanoma is generally very poor. PATIENT: The authors describe the case of a 29-year-old woman with unilateral hearing loss and facial paresis. Magnetic resonance imaging (MRI) demonstrated a mass that was thought to be an acoustic neuroma but was seen to involve the cochlea as well as the internal auditory meatus and cerebellopontine angle. The lesion was subsequently excised completely by a trans-labyrinthine approach, with facial nerve preservation, and was shown on histologic examination to be a malignant melanoma. Further comprehensive investigation did not reveal a primary extracranial site or any sign of CNS spread. The clinical features of this case, including the radiologic and histologic findings, are described, and literature concerning management is reviewed.