Primary Middle Ear Mucosal Melanoma: Case Report and Comprehensive Literature Review of 21 Cases of Primary Middle Ear and Eustachian Tube Melanoma.

OBJECTIVE: To present a case of primary middle ear mucosal melanoma and perform a comprehensive literature review of middle ear and eustachian tube mucosal melanoma.

PATIENT: A 61-year-old female presented with no prior history of melanoma and 3 months of aural fullness. A middle ear mass demonstrated primary mucosal melanoma. The mass extended from mesotympanum into hypotympanum, epitympanum, protympanum, eustachian tube, and mastoid antrum. Additionally, a nonenhancing expansile lesion of the petrous apex was noted on magnetic resonance imaging.

INTERVENTION: Subtotal temporal bone resection with transotic approach to the petrous abnormality was performed. Postoperative adjuvant radiation and immunotherapy were given.

RESULTS: Five months postoperatively, the patient developed an isolated cutaneous metastatic focus treated with wide local excision and has had no further evidence of recurrence to date. Upon comprehensive literature review, patients with primary middle ear melanomas (n = 10) present with otorrhea (50%), aural fullness (40%), and hearing loss (30%) most commonly, while hearing loss (81.8%) and aural fullness (54.5%) were the most common presenting symptoms for eustachian tube melanomas (n = 11). Patients were treated with combinations of surgery, radiation, and/or chemotherapy. Middle ear melanoma demonstrated particularly poor outcomes, with 70% mortality, 20% local recurrence, and 50% distant metastasis, whereas eustachian tube origin demonstrated 9.1%, 18.2%, and 36.4%, respectively.

CONCLUSIONS: Middle ear and eustachian tube mucosal melanomas are exceedingly rare, with middle ear melanomas demonstrating a worse prognosis. Multimodality therapy is commonly used; however, outcomes are poor, with high mortality among affected patients.