ABSTRACT
Familial colorectal adenocarcinoma (CRC) accounts for ~15 to 20% of CRC. Of these,
hereditary nonpolyposis colorectal cancer (HNPCC) and familial adenomatous polyposis
(FAP) represent the most common hereditary syndromes associated with CRC, followed
by other less common diseases including juvenile polyposis (JP) and Peutz–Jeghers
syndrome (PJS). Extracolonic manifestations are common in each of these syndromes
having significant implications for surveillance and management in at-risk individuals.
The authors review the most common and clinically relevant extracolonic manifestations
for each of these syndromes focusing on incidence, presentation, genotype/phenotype
correlations, and management (including surveillance) strategies.
KEYWORDS
Hereditary polyposis - colorectal cancer - extracolonic manifestations
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George J ChangM.D.
Department of Surgical Oncology, The University of Texas M. D. Anderson Cancer Center
1515 Holcombe Blvd., Unit 444, P.O. Box 301402, Houston, TX 77230
eMail: gchang@mdanderson.org
