title: Primary ear and temporal bone meningiomas: a clinicopathologic study of 36 cases with a review of the literature.
abstract: "Primary" ear and temporal bone meningiomas are tumors that are frequently misdiagnosed and unrecognized, resulting in inappropriate clinical management. To date, a large clinicopathologic study of meningiomas in this anatomic site has not been reported. Thirty-six cases of ear and temporal bone meningiomas diagnosed between 1970 and 1996 were retrieved from our files. Histologic features were reviewed, immunohistochemical analysis was performed (n = 19), and patient follow-up was obtained (n = 35). The patients included 24 females and 12 males, aged 10-80 years (mean, 49.6 years), with female patients presenting at an older age (mean, 52.0 years) than male patients (mean, 44.8 years). Patients presented clinically with hearing changes (n = 20), otitis (n = 7), pain (n = 5), and/or dizziness/vertigo (n = 3). Symptoms were present for an average of 24.6 months. The tumors affected the middle ear (n = 25), external auditory canal (n = 4), or a combination of temporal bone and middle ear (n = 7). The tumors ranged in size from 0.5 to 4.5 cm in greatest dimension (mean, 1.2 cm). Radiographic studies demonstrated a central nervous system connection in 2 patients. Histologically, the tumors demonstrated features similar to those of intracranial meningiomas, including meningothelial (n = 33), psammomatous (n = 2), and atypical (n = 1). An associated cholesteatoma was identified in 9 cases. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (79%) and vimentin (100%). The differential diagnosis includes paraganglioma, schwannoma, carcinoma, melanoma, and middle ear adenoma. Surgical excision was used in all patients. Ten patients developed a recurrence from 5 months to 2 years later. Five patients died with recurrent disease (mean, 3.5 years), and the remaining 30 patients were alive (n = 25, mean: 19.0 years) or had died (n = 5, mean: 9.5 years) of unrelated causes without evidence of disease. We conclude that extracranial ear and temporal bone meningiomas are rare tumors histologically similar to their intracranial counterparts. They behave as slow-growing neoplasms with a good overall prognosis (raw 5-y survival, 83%). Extent of surgical excision is probably the most important factor in determining outlook because recurrences develop in 28% of cases.
authors.affiliation: Department of Endocrine and Otorhinolaryngic-Head & Neck Pathology, Armed Forces Institute of Pathology, Washington, DC 20306, USA.
authors.forename: Lester D R
authors.initials: LD
authors.lastname: Thompson
authors.forename: John-Paul
authors.initials: JP
authors.lastname: Bouffard
authors.forename: Glenn D
authors.initials: GD
authors.lastname: Sandberg
authors.forename: Hernando
authors.initials: H
authors.lastname: Mena
mesh_terms: D000236:Adenoma / Q000473:pathology; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002277:Carcinoma / Q000473:pathology; D002648:Child; D003937:Diagnosis, Differential; D004428:Ear Neoplasms / Q000378:metabolism / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008545:Melanoma / Q000473:pathology; D008577:Meningeal Neoplasms / Q000378:metabolism / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology; D008579:Meningioma / Q000378:metabolism / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D009442:Neurilemmoma / Q000473:pathology; D010235:Paraganglioma / Q000473:pathology; D011379:Prognosis; D013701:Temporal Bone / Q000378:metabolism / Q000473:pathology*