Recurrent glomangioma (“true” glomus tumor) of the middle ear and mastoid

OBJECTIVE: To review current literature and experience with glomangiomas, or true glomus tumors of the middle ear and mastoid as well asto report on the exceptionally rare case of a glomangiomastemming from the middle ear space with multiple recurrences. METHODS: Review of existing world literature and description of personal experience with rare cases of a glomangioma of the middle ear and mastoid. RESULTS: Review of existing literature revealed two cases of patients presenting with tinnitus and hearing loss refractory to medical management. Both patients were ultimately diagnosed with glomangioma on histopathology. Complete surgical excision is thought to be curative. PATIENT: A 36-year-old woman presented with a rare case of a glomangioma of the middle ear presenting with unilateral hearing loss. She was noted to have a mass behind the tympanic membrane. Imaging revealed a diffuse mass filling the mastoid air cells. Imaging characteristics and histology were consistent with a glomangioma. INTERVENTION: Initial resection via mastoidectomy using a postauricular approach. The tympanic membrane was reconstructed with temporalis tissue. Follow-up revision tympanomastoidectomy was performed upon recurrence of disease. The chorda tympani were sacrificed due to tumor involvement. The incus and head of the malleus were removed to gain better access to the tumor. The ossicular chain was reconstructed with a Goldenberg Total Ossicular Prosthesis. MAIN OUTCOME MEASURE: Recurrence of disease. FOLLOW-UP: In the 67 months since her most recent surgery, there has been no evidence of recurrence by CT or physical exam. CONCLUSION: Glomangioma of the middle ear represents an exceptionally rare entity that can present in a similar fashion to a paraganglioma.