Synonyms
Cylindroma
Definition
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.
Macroscopy
The most common macroscopic presentation pattern is as an intraluminal, polypoid mass. Four...
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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.
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.
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.
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.
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Fonseca, I. (2017). Adenoid Cystic Carcinoma, Esophageal. In: Carneiro, F., Chaves, P., Ensari, A. (eds) Pathology of the Gastrointestinal Tract. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-40560-5_1556
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DOI: https://doi.org/10.1007/978-3-319-40560-5_1556
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