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Clinical Challenge
March 2016

A Mastoid Mass With Associated Pulmonary Cystic Lesions

Author Affiliations
  • 1Department of Otolaryngology–Head Neck Surgery, Kaiser Permanente Oakland Medical Center, Oakland, California
  • 2Department of Otolaryngology, Tufts Medical Center, Boston, Massachusetts

Copyright 2016 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.

JAMA Otolaryngol Head Neck Surg. 2016;142(3):295-296. doi:10.1001/jamaoto.2015.3246

A previously healthy young woman presented to the emergency department with right-sided otalgia of several weeks’ duration and was treated with amoxicillin and dexamethasone-ciprofloxacin otic drops. One week later she re-presented with worsening right-sided otalgia and intermittent rotary vertigo. On physical examination she was noted to have protrusion of the right auricle and marked right mastoid erythema and tenderness. She was afebrile and denied otorrhea, tinnitus, hearing loss, headache, or shortness of breath. Further questioning elicited a history of amenorrhea. The patient had had several episodes of otitis media as a child and had been smoking cigarettes for 4 years but had no other significant medical history, no surgical history, or family history of otologic disease. Computed tomographic (CT) imaging of the head and neck revealed an expansile mass in the right mastoid cavity with associated destruction of the adjacent cortical bone and a markedly narrowed right sigmoid sinus (Figure, A). A CT image of the chest demonstrated multiple bilateral cystic lesions and reticular nodular densities with an apical to basal gradient (Figure, B). T1-weighted magnetic resonance imaging of the brain demonstrated heterogeneous enhancement of the pituitary gland and thickening of the infundibulum (Figure, C). A skeletal survey was otherwise negative for abnormalities. Pathologic findings from a biopsy specimen of the right mastoid lesion revealed sheets of immature dendritic cells in association with prominent eosinophils. Immunohistochemical staining indicated that the neoplastic cells were positive for CD1a.

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