Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Adenoid Cystic Carcinoma, Esophageal

  • Isabel Fonseca
Reference work entry




Adenoid cystic carcinoma is a basaloid tumor consisting of epithelial and myoepithelial cells (WHO) in variable architectural configurations, including tubular, cribriform, and solid patterns.

Clinical Features

  • Incidence

    Adenoid cystic carcinoma is extremely rare in the esophagus, accounting for less than 0.1% of esophageal carcinomas.

  • Age

    Most patients are in their seventh decade.

  • Sex

    Males are more commonly affected with a reported M-F ratio of 3:1.

  • Site

    More than half of the cases occur in the middle third of the esophagus. They rarely arise in the upper third.

  • Treatment

    Surgery is the treatment of choice. In cases with positive margins, radiotherapy is warranted.

  • Outcome

    The prognosis is poor, usually fatal. However, some cases have slow progression over large periods of time. Recurrence is found in regional lymph nodes as well as distant organs.


The most common macroscopic presentation pattern is as an intraluminal, polypoid mass. Four...

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References and Further Reading

  1. El-Naggar, A. K., & Huvos, A. G. (2005). Adenoid cystic carcinoma. In L. Barnes, J. W. Eveson, P. Reichart, & D. Sidransky (Eds.), World Health Organization classification of tumours. Pathology and genetics of head and neck tumours (pp. 221–222). Lyon: IARC Press.Google Scholar
  2. Lieberman, M. D., Franceschi, D., Marsan, B., & Burt, M. (1994). Esophageal carcinoma. The unusual variants. The Journal of Thoracic and Cardiovascular Surgery, 108, 1138–1146.PubMedGoogle Scholar
  3. Morisaki, Y., Yoshizumi, Y., Hiroyasu, S., Shibata, H., Terahata, S., Tamai, S., Sugiura, Y., Shima, S., & Tanaka, S. (1996). Adenoid cystic carcinoma of the esophagus: Report of a case and review of the Japanese literature. Surgery Today: The Japanese Journal of Surgery, 26, 1006–1009.Google Scholar
  4. Persson, M., Andrén, Y., Mark, J., Horlings, H. M., Persson, F., & Stenman, G. (2009). Recurrent fusion of MYB and NFIB transcription factor genes in carcinomas of the breast and head and neck. Proceedings of the National Academy of Sciences of the United States of America, 106, 18740–18744.PubMedPubMedCentralGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Serviço de Anatomia PatológicaInstituto Português de Oncologia Francisco Gentil – LisboaLisbonPortugal
  2. 2.Faculdade de Medicina de LisboaInstituto de Anatomia PatológicaLisbonPortugal