2005 EDITORIALS: It is Time to Get Serious About Diagnosing Lynch Syndrome

"Although patient-reported family cancer histories appear
to be accurate and valuable for colorectal cancer risk
assessments, nearly one half of oncologists fail to document
a comprehensive family history, and among those
that do, few take appropriate action if it is positive with
respect to referral for genetic evaluation."

Hereditary nonpolyposis colorectal cancer (HNPCC),
also called Lynch syndrome after Henry T. Lynch,
MD, a pioneer in the field, is an autosomal dominant
hereditary cancer syndrome, which accounts for upwards
of 3% of all colorectal cancers and is associated with
an increased risk of endometrial, ovarian, and other extracolonic
cancers. Colorectal cancer can be averted in
Lynch syndrome by early an intensive surveillance, and
has been shown to be cost-effective. The syndrome
originally was defined in clinical terms by the stringent
Amsterdam criteria, although over time more relaxed
clinical definitions have been suggested, culminating in
the recently published revised Bethesda guidelines.