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

Abstract

ObjectiveTo 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.MethodsReview of existing world literature and description of personal experience with rare cases of a glomangioma of the middle ear and mastoid.ResultsReview 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.PatientA 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.InterventionInitial 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 measureRecurrence of disease.Follow‐upIn the 67 months since her most recent surgery, there has been no evidence of recurrence by CT or physical exam.ConclusionGlomangioma of the middle ear represents an exceptionally rare entity that can present in a similar fashion to a paraganglioma.

A rare case of a middle ear glomangioma

“…This is the fourth reported case of a middle ear glomangioma, all of which have occurred in females and been further classified as glomangiomas. 6 – 8 Our patient presented similarly to previous cases with pulsatile tinnitus, ear fullness, and objective hearing loss (see Table 1 ). She later developed otalgia.…”

“…However, the tumors are not encapsulated, and it is possible to have tumor recurrence if not completely excised. 6 True glomus tumors are rare in the head and neck. This is the fourth reported case of a middle ear glomangioma, all of which have occurred in females and been further classified as glomangiomas.…”

“…Unlike glomus tympanicum tumors, glomangiomas stain negatively for neuroendocrine markers such as chromogranin A, S100, and synaptophysin. 6 – 8 Instead, pathology shows positive expression of smooth muscle actin (SMA). 7 , 8 In our case, immunochemistry was not performed due to the characteristic appearance of the specimen.…”

“… 4 , 5 The middle ear is an even more exceptionally rare location for these tumors with only three cases reported in the literature to date. 6 We report a case of a middle ear glomangioma, which is the fourth reported case of a glomangioma in this location.…”

Glomangioma of the External Ear: A Case Report

“…Glomus tumors are rare soft tissue neoplasms of mesenchymal origin that result from undifferentiated smooth muscle (glomus) cell hyperplasia, similar to what is found in the normal glomus body. 1,2 Glomus bodies are cutaneous structures that consist of neuromyoarterial cells and play a role in thermoregulation. They are specifically found in the dermal layer of skin, most commonly in the hands and feet.…”