Primary Melanocytic Tumor of the Cerebellopontine Angle Mimickinga Vestibular Schwannoma

Abstract

The majority of tumors of the cerebellopontine angle (CPA) are benign. We report the case of a primary malignant melanoma of the CPA that mimicked a vestibular schwannoma (acoustic neuroma). We discuss the differential diagnosis and prognosis of melanotic lesions at this location. A 49-year-old man presented with a 7-year history of unilateral deafness and a several month history of imbalance, intractable nausea and vomiting, as well as weight loss. A neurological work-up revealed a large tumor in the left CPA radiographically diagnosed as a vestibular schwannoma. A translabyrinthine approach revealed a pigmented, vascular neoplasm encasing vessels and cranial nerves of the left CPA. The tumor was subtotally resected, and a histopathological diagnosis of melanoma was made. The patient had no history of cutaneous melanoma and no other site of disease was ever discovered. This case most likely represents primary melanoma of the central nervous system that mimicked a vestibular schwannoma. The differential diagnosis of melanotic lesions of the CPA is discussed as are the prognostic implications of each diagnosis.