Salivary glands
Epithelial / myoepithelial tumors
Clear cell carcinoma

Author: Adriana Handra-Luca, M.D. (see Authors page)

Revised: 15 March 2018, last major update September 2012

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Clear cell carcinoma[TI] salivary

See also: Hyalinizing clear cell carcinoma
Cite this page: Handra-Luca, A. Clear cell carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/salivaryglandsclearcellcarcinomasalivary.html. Accessed June 18th, 2018.
Definition / general
  • First described in 1975 by Mohamed (Cancer 1975;36:1057) and in 1983 by Chen (Hum Pathol 1983;14:91)
  • < 100 cases reported to date
  • May be a type of squamous cell carcinoma (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;108:399)
  • Varies from monomorphic tumor (with or without hyalinizing stroma) with squamous, glandular or no differentiation to monomorphic clear cell myoepithelial carcinoma to bimorphic epithelial myoepithelial carcinoma (adenomyoepithelioma, glycogen rich adenoma and glycogen rich adenocarcinoma)
Clinical features
  • Usually adult women with painless mass; 91% in minor salivary gland tumors (palate, base of the tongue)
  • 1% of intraoral minor salivary gland tumors; also occur in major salivary glands
  • Usually age 30+ years
  • May occur in pleomorphic adenoma (Acta Cytol 2006;50:687), be associated with HIV (Int J Oral Maxillofac Surg 2011;40:760)
  • Low grade but also aggressive
  • 19% lymph node positive, 8% distant metastases, 3% have death due to disease
Case reports
Treatment
Gross description
  • Infiltrating, scar-like
Microscopic (histologic) description
  • Trabeculae, cords, islands or nests of monomorphic clear cells
  • Also cells with eosinophilic and granular cytoplasm
  • Infiltrative borders
  • Variable atypia
  • No / rare mitotic figures
  • No myoepithelial differentiation
Microscopic (histologic) images

Images hosted on other servers:

Various images

Electron microscopy description
  • Abundant glycogen (Ultrastruct Pathol 1996;20:519), desmosomes, peripheral tonofilaments, squamoid differentiation, prominent interdigitating microvilli without actin myofilaments or dense bodies
  • No lipid, no zymogen granules, no true ductal lumina, no myoepithelial differentation
Electron microscopy images

Images hosted on other servers:

Various images

Differential diagnosis