Encyclopedia of Radiation Oncology

2013 Edition
| Editors: Luther W. Brady, Theodore E. Yaeger

Familial Adenomatous Polyposis (FAP)

Reference work entry
DOI: https://doi.org/10.1007/978-3-540-85516-3_215

Definition

This is a rare disorder resulting from dysregulation of the APC protein either through direct genomic errors or indirectly through proteins which mediate the function of the APC protein. Classically, FAP displays autosomal dominant inheritance. The disorder has mixed penetrance with 95% patients displaying polyps by age 35 years while some suffer from hundreds of colonic polyps in childhood.

This is an inherited syndrome in which numerous polyps form mainly in the epithelium of the large intestine. While these polyps start out benign, malignant transformation into colorectal cancer occurs when not treated.

Cross-References

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  1. 1.Department of Radiation OncologyPhiladelphiaUSA
  2. 2.Department of Radiotherapy and Radiation OncologyUniversity Hospital Frankfurt/MainFrankfurtGermany