Middle ear and inner ear tumors - benign / nonneoplastic
Middle ear paraganglioma

Topic Completed: 1 April 2015

Minor changes: 6 November 2019

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PubMed Search: paraganglioma [title] middle ear

Nat Pernick, M.D.
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Cite this page: Pernick N. Middle ear paraganglioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/earjugulotympanicparaganglioma.html. Accessed September 24th, 2020.
Definition / general
  • Also called glomus jugulare tumor or glomus tympanicum tumor
  • Most common tumor of middle ear
  • Usually women, ages 40 - 69 years
  • 85% arise in jugular bulb causing mass in middle ear or external auditory canal; 12% arise from tympanic branch of glossopharyngeal nerve (Jacobson nerve) causing middle ear mass; 3% arise from posterior auricular branch of vagus nerve (Arnold nerve) causing external auditory canal mass
  • Usually causes conductive hearing loss
  • May be locally invasive into temporal bone and mastoid; may cause cranial nerve palsies, cerebellar dysfunction, dysphagia, hoarseness
  • Tumors are fed by branches of nearby large arteries; may bleed profusely at biopsy
  • Histology usually benign but this does not predict behavior
  • Rarely are malignant histologically (necrosis, mitotic activity, vascular invasion) with metastases to cervical lymph nodes, lung, liver (J Laryngol Otol 2000;114:17)
Case reports
  • Complete excision (may be difficult) with possible preoperative embolization or radiation therapy (reduces vascularity, promotes fibrosis)
  • 50% recur locally
Gross description
  • Polypoid, red, friable
Microscopic (histologic) description
  • Classic organoid (zellballen) or nesting pattern of paragangliomas with central round / oval chief cells containing abundant eosinophilic granular or vacuolated cytoplasm, uniform nuclei with dispersed chromatin
  • Sustentacular cells (spindled, basophilic, difficult to see with H&E) are present at periphery of nests
  • Prominent fibrovascular stroma separates nests
  • May have pleomorphism but this does not predict malignant behavior; occasional dense fibrous stroma or apparent infiltrative growth
  • Rare mitotic figures or necrosis
  • No glandular or alveolar differentiation
Microscopic (histologic) images

Images hosted on PathOut server:

Contributed by Kelly Magliocca D.D.S., M.P.H.

Middle ear paraganglioma, 4x

Middle ear paraganglioma, Zellballen

Synaptophysin positive in chief cells, 20x

S100 in sustentacular pattern, 40x

Case of the Week #349
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H&E images

Positive stains
Negative stains
Electron microscopy description
  • Neurosecretory granules
Molecular / cytogenetics description
Differential diagnosis
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