Middle ear, inner ear and temporal bone tumors - malignant
Aggressive papillary tumor

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

Revised: 20 February 2018, last major update October 2013

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

PubMed Search: Aggressive papillary tumor ear

Cite this page: Pernick, N. Aggressive papillary tumor . PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/earendolymphaticsac.html. Accessed April 27th, 2018.
Definition / general
  • Also called papillary adenoma of endolymphatic sac / temporal bone, adenocarcinoma of temporal bone / mastoid, endolymphatic sac tumor, low grade adenocarcinoma of probable endolymphatic sac origin, Heffner tumor
  • Uncommon, associated with von Hippel-Lindau syndrome (11% have these tumors) and female adnexal tumor of presumed wolffian origin (Am J Surg Pathol 1994;18:1254); also somatic mutations of VHL gene in non VHL patients (Cancer Res 2000;60:5963)
  • Most arise within intraosseous portion of the endolymphatic duct / sac, with precursor lesions present in VHL patients (Cancer Res 2005;65:10847)
  • Median age 30s, range of 11 - 71 years
  • Symptoms: early sensorineural hearing loss, tinnitus and episodic vertigo
Radiology description
  • Tumor in posterior medial petrous ridge of temporal bone (site of endolymphatic sac)
Case reports
  • Radical surgery including mastoidectomy and temporal bone resection with possible loss of cranial nerves
  • Tumor grows slowly with only one reported metastasis (J Neurosurg Spine 2005;3:68) but may recur with inadequate excision
  • Is infiltrative, destructive and may cause death
  • Bleeds profusely at surgery
Microscopic (histologic) description
  • Simple papillary structures composed of single layer of columnar to cuboidal epithelium, often with distinct cell boundaries
  • May have apparent myoepithelial layer that actually is flattened stroma; epithelial cells have pale-clear cytoplasm, uniform central or luminal nuclei
  • Often granulation tissue reaction with small vascular spaces and mixed inflammatory infiltrate is present next to tumor cells
  • Occasional thyroid like hypercellular areas with cystic glandular spaces containing colloid like material
  • May have areas of recent hemorrhage with cholesterol clefts
  • Minimal pleomorphism
  • No / rare mitotic figures or necrosis
  • Resembles choroid plexus papilloma
Microscopic (histologic) images

Images hosted on other servers:
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Various images

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Papilla with hemorrhagic material

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Papilla with vacuolated cells and colloid-like material

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A: papillae lined by bland cuboidal cells extending into bone
B: tumor cells have moderate cytoplasm, round/oval bland
nuclei, finely granular chromatin, indistinct nucleoli;
C: EMA+; D: CK7+

Cytology description
  • Rare epithelial cell clusters, some with papillary features
  • Foamy macrophages
  • Epithelial cells have eosinophilic and focally vacuolated cytoplasm, some with pigmented granules resembling hemosiderin, well defined cell borders, bland nuclei
Positive stains
Negative stains
Electron microscopy description
  • Intercellular junctional complexes, microvilli, basement membrane, rough ER, glycogen, secretory granules
Differential diagnosis