Middle ear, inner ear and temporal bone tumors - benign / nonneoplastic
Jugulotympanic paraganglioma

Author: Nat Pernick, M.D. (see Authors page)

Revised: 23 February 2018, last major update April 2015

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Jugulotympanic paraganglioma [title]

Cite this page: Pernick, N. Jugulotympanic paraganglioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/earjugulotympanicparaganglioma.html. Accessed March 24th, 2018.
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

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Case of the Week #349:
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H&E images

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Bladder tumor: H&E and S100

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