Skip to main content

Table 1 ACN Guidelines

From: Survey of HNPCC Management Analysis of Responses from 18 International Cancer Centres

The Australian Cancer Network (2002) has developed guidelines for categorizing families, and recommends referral of families for consideration of risk assessment and pre-genetic or genetic testing to familial cancer services.

Suggested Definition of "Potentially High Risk":

• Three or more first- or second-degree relatives on the same side of the family are diagnosed with bowel cancer.

• Two or more first- or second-degree relatives on the same side of the family are diagnosed with bowel cancer plus any of the following high risk features:

   - multiple bowel cancers in a family member,

   - bowel cancer before age 50 years,

   - a family member who has/had an HNPCC-related cancer (endometrial, ovarian, stomach, small bowel, renal pelvis or ureter, biliary tract, brain cancer)*

• at least one first- or second-degree relative diagnosed with bowel cancer with a large number of synchronous adenomas (suspected FAP),

• there is a member of the family in which a gene mutation that confers a high risk of bowel cancer has been identified.

*The Australian Cancer Network guidelines extend the range of cancers implicated in HNPCC based on subsequent literature identifying other cancers in the syndrome.

Additional tumours for consideration: Breast in hMLH1 families [1], clear cell kidney [2].

Suggested Definition of "Moderately Increased Risk":

• One first-degree relative with bowel cancer diagnosed before age 55 years (without potentially high-risk features).

• Two first- or second-degree relatives on the same side of the family with bowel cancer diagnosed at any age (without potentially high-risk features).