Clin Colon Rectal Surg 2008; 21(4): 263-272
DOI: 10.1055/s-0028-1089941
© Thieme Medical Publishers

Extracolonic Manifestations of Hereditary Colorectal Cancer Syndromes

Daniel A. Anaya1 , George J. Chang1 , Miguel A. Rodriguez-Bigas1
  • 1Department of Surgical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas
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Publication History

Publication Date:
14 October 2008 (online)

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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.

REFERENCES

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