Ear
Middle ear and inner ear tumors - benign / nonneoplastic
Middle ear paraganglioma
Author: Nat Pernick, M.D.
Last author update: 1 April 2015
Last staff update: 19 November 2024 (update in progress)
Copyright: 2002-2025, PathologyOutlines.com, Inc.
PubMed Search: paraganglioma middle ear
Table of Contents
Definition / general | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosis | Additional referencesCite this page: Pernick N. Middle ear paraganglioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/earjugulotympanicparaganglioma.html. Accessed January 3rd, 2025.
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
- 45 year old man with middle ear mass (Case of the Week #349)
- Patient with tumor with regional metastases and spinal metastases 10 - 13 years after presentation (Arch Pathol Lab Med 1990;114:976)
Treatment
- 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
H&E images
Positive stains
- Chromogranin and synaptophysin (chief cells), S100 (sustentacular cells)
- Reticulin (stains stroma and delineates nesting pattern, particularly helpful with crushed specimens), variable vimentin (both cell types)
Electron microscopy description
- Neurosecretory granules
Molecular / cytogenetics description
- Germline mutations for succinate dehydrogenase gene subunits if multiple tumors (Diagn Mol Pathol 2005;14:109)
Differential diagnosis
- Acoustic neuroma
- Carcinoma
- Melanoma
- Meningioma
- Middle ear adenoma
- Other neuroendocrine tumors
Additional references
