Primary ear and temporal bone meningiomas: a clinicopathologic study of 36 cases with a review of the literature

Mod Pathol. 2003 Mar;16(3):236-45. doi: 10.1097/01.MP.0000056631.15739.1B.

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.

MeSH terms

  • Adenoma / pathology
  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Carcinoma / pathology
  • Child
  • Diagnosis, Differential
  • Ear Neoplasms / metabolism
  • Ear Neoplasms / mortality
  • Ear Neoplasms / pathology*
  • Ear Neoplasms / physiopathology
  • Female
  • Follow-Up Studies
  • Humans
  • Immunohistochemistry
  • Male
  • Melanoma / pathology
  • Meningeal Neoplasms / metabolism
  • Meningeal Neoplasms / mortality
  • Meningeal Neoplasms / pathology*
  • Meningeal Neoplasms / physiopathology
  • Meningioma / metabolism
  • Meningioma / mortality
  • Meningioma / pathology*
  • Meningioma / physiopathology
  • Middle Aged
  • Neoplasm Recurrence, Local
  • Neurilemmoma / pathology
  • Paraganglioma / pathology
  • Prognosis
  • Temporal Bone / metabolism
  • Temporal Bone / pathology*