Adenoid cystic carcinoma

Topic Completed: 1 August 2013

Minor changes: 15 December 2020

Copyright: 2003-2021,, Inc.

PubMed Search: Adenoid cystic carcinoma esophagus

Elliot Weisenberg, M.D.
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Cite this page: Weisenberg E. Adenoid cystic carcinoma. website. Accessed October 22nd, 2021.
Definition / general
  • Primary tumor of esophagus morphologically and immunohistochemically identical to adenoid cystic carcinoma of salivary glands
  • Believed to arise from esophageal glands
  • Very rare, generally middle age, more common in females
Prognostic factors
  • Generally favorable prognosis with excellent survival
  • Superior to squamous cell carcinoma and usual adenocarcinoma of the esophagus
Case reports
Gross description
  • Generally well circumscribed nodule in submucosa
Microscopic (histologic) description
  • Identical to tumor in salivary gland
  • Inner ductal type epithelium and outer modified myoepithlial cells form solid nests or cribriform spaces containing balls of glyocosaminoglycans and basement membrane material
Microscopic (histologic) images

Contributed by Dr. Mark R. Wick

Adenoid cystic carcinoma

Positive stains
  • Ductal epithelium is strongly cytokeratin and CEA+, while modified myoepithelial cells are weakly cytokeratin positive with strong S100, actin and vimentin positivity in modified myoepithelial cells
Differential diagnosis
  • Basaloid squamous cell carcinoma
    • More pleomorphic with greater mitotic activity
    • Does not form true lumina and lacks CEA staining
    • Generally CK19+, not seen in adenoid cystic carcinoma
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