Remission of Mucosal Melanoma of the Middle Ear and Petrous Temporal Bone and Reversal of Cranial Nerve Paresis Following Radiation and Single Agent Nivolumab: Clinical Capsule and Review of the Literature

Abstract

Objective: We report disease remission and recovery of fifth and seventh nerve paresis in a case of primary mucosal melanoma of the middle ear and petrous temporal bone. Patient: A 74-year-old man developed sudden, profound, right sided sensorineural hearing loss, disequilibrium, otalgia, and cranial nerve V and VII dysfunction. Imaging demonstrated an unresectable, osteolytic lesion involving the middle ear and anterior petrous apex. Melanoma was diagnosed via in-office biopsy; whole-body metabolic imaging revealed no other primary site. Intervention: Multidisciplinary management included radiation therapy (30 Gy, 10 fractions) followed by induction (five cycles, q2w) and maintenance nivolumab (six cycles, q3w). Main Outcome Measure: Complete metabolic response of primary site and metastases on imaging, recovery of cranial neuropathies.Results: Following palliative radiation therapy and induction nivolumab, cranial neuropathies resolved. With maintenancedose nivolumab, primary site and metastases exhibited a complete response. Therapy was stopped at 16 months postdiagnosis. Complete remission was maintained until 22 months after diagnosis. The patient developed a solitary cerebral metastasis which was refractory to radiosurgery and biopsy confirmed melanoma. He expired 2 years, 8 months post-diagnosis. Conclusions: Mucosal melanoma of the middle ear and petrous temporal bone is exceedingly rare. Management is individualized and surgery is undertaken when possible. Key observations in this case are the complete metabolic response and reversal of cranial nerve neuropathies following radiation and anti-programed cell death receptor ligand 1 therapy. Non-surgical treatment is worthy of study as initial management for similar lesions.

A Systematic Review of Primary Temporal Bone Mucosal Melanoma

“…Nevertheless, physicians should include temporal bone MM as a potential diagnosis in patients presenting with bloody otorrhea in the context of a chronic, recurrent serous otitis media, as observed in several of the patients in the current review (22,(24)(25)(26)(27)(28). Furthermore, CN V, VI, and VII palsies should indicate the need for immediate further workup for potential temporal bone disease (8,20,21,(28)(29)(30)(31). Recognizing the aforementioned symptoms earlier may promote the timely diagnosis of temporal bone MM, which has historically been one of the largest obstacles in improving patient outcomes (32).…”

“…However, of these head and neck MMs, there have been few reported cases originating from the middle ear or eustachian tube (7). These malignancies can erode through the temporal bone, inducing cranial nerve (CN) impairment, hearing loss, vertigo, or otalgia (8). Because of the rarity, insidious nature, and nonspecific presentation of temporal bone MM, patients may experience delays in diagnosis and treatment, as this condition may be initially misdiagnosed as serous otitis media or otomastoiditis (9)(10)(11).…”