Middle ear and inner ear tumors - benign / nonneoplastic

Middle ear paraganglioma

Last author update: 1 April 2015
Last staff update: 13 May 2022

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PubMed Search: paraganglioma middle ear

Nat Pernick, M.D.
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Cite this page: Pernick N. Middle ear paraganglioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/earjugulotympanicparaganglioma.html. Accessed June 3rd, 2023.
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

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 #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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