Otolaryngology Case Reports (Sep 2020)
More than meets the eye: A reportedly isolated ear canal mass
Abstract
Background: Peripheral nerve sheath myxomas are benign tumors which primarily occur on the central face, arms, and shoulders. Here we present a rare case of nerve sheath myxoma in the external auditory canal (EAC) and review the differential diagnosis, relevant histopathological characteristics of this tumor, and considerations for management including workup for the associated Carney complex, an autosomal dominant disorder characterized by cardiac myxomas, endocrine tumors involving the thyroid and pituitary gland, and melanotic schwannomas. Case description: A healthy 18-year-old male presented with an isolated soft-tissue mass in the right EAC. Otoscopic examination revealed a subepithelial 1.5‐cm soft-tissue mass within the lateral anterosuperior EAC. Audiometric testing for the right ear demonstrated a mild low frequency conductive loss with type C tympanogram. Contrasted computed tomography of the temporal bones revealed an ill-defined partially exophytic soft tissue density/mass in the right anterior lateral EAC. The patient subsequently underwent wide local excision and partial ear canal sleeve resection. Final pathology revealed peripheral nerve sheath myxoma and appropriate genetic and endocrine workup for Carney complex was performed. Conclusions: This case highlights the value of thorough diagnosis and management of neoplasms of the EAC. Nerve sheath myxoma is an important consideration for otolaryngologists when evaluating EAC lesions given the risk of systemic involvement with high associated morbidity when associated with Carney complex. Appropriate syndromic workup and close follow-up for significant risk of recurrence is warranted.
