Hereditary nonpolyposis colorectal cancer (HNPCC), often called Lynch syndrome, may be described as a hereditary predisposition to developing colorectal and extracolonic cancers. Accounting for approximately 3% of all colorectal malignancies, it is the most common cause of hereditary colorectal cancer [
]. The diagnosis is based on clinical criteria related to family history of certain HNPCC-defining cancers, such as those of the colorectum, uterus, stomach, ovaries, urinary epithelium, and small bowel. The syndrome is characterized by early onset of cancer, and an elevated clinical suspicion is needed to make a timely diagnosis so that appropriate surveillance and intervention can be performed to decrease deaths from cancer. This review provides a brief background on HNPCC and reviews the surgical management of the disease.
- Hampel H.
- Frankel W.L.
- Martin E.
- et al.
Feasibility of screening for Lynch syndrome among patients with colorectal cancer.
J Clin Oncol. 2008; 26: 5783-5788
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Published online: July 04, 2011
© 2011 Published by Elsevier Inc.