Ms. Greco's excellent review of the hereditary forms of colorectal cancer points out the importance of oncology nurses' understanding of the syndromes that may affect patients in their practices. As the field of genomics continues to expand with direct applicability to oncology care, nurses working with oncology patients need to be aware of the type and incidence of hereditary cancer syndromes, including colorectal cancer syndromes.

Colorectal cancer is an extremely common cancer, affecting 5% to 6% of the general population; however, patients with a familial risk (those who have two or more relatives with a first- or second-degree relative with a diagnosis of cancer) actually make up almost 20% of the colorectal cancer cases.[1] Five to ten percent are inherited in an autosomal dominant manner, including hereditary, familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer.[1]

Patient Counseling

When counseling patients with hereditary colorectal cancer syndromes, careful attention must be paid to education, support and counseling for all family members. This is especially important with the sometimes daunting diagnosis of famililal polyposis syndrome (FAP) in a young person. Family members may be understandably distraught when receiving the news that a 15-year-old has colorectal cancer and must undergo colectomy; however, the importance of having all additional family members receive screening cannot be overemphasized. A comprehensive and detailed family history of malignancy is essential to identify a true hereditary colorectal cancer with subsequent molecular testing to verify the syndrome.[2]

Once identified, appropriate intervention and surveillance are critical to early diagnosis and improved outcome. In some cases, chemoprevention may be helpful, such as with FAP patients in one study that experienced a reduction in the number of colorectal polyps after treatment with celecoxib twice a day for 6 months vs the placebo group.[3] Although the polyps may return, this approach may delay the need for a prophylactic colectomy, however careful follow-up is essential to determine the patient's status and need for surgery in this extremely high-risk group.

Legal Implications

Lastly, the legal implication for a new diagnosis of hereditary cancer syndrome is a point of concern. Although many insurance companies have coverage for predisposition testing of cancer, there may be concerns regarding release of positive results to the insurance company due to fear of insurance cancellation or rate increases.[4] Therefore, it is crucial that oncology nurses working with patients undergoing testing for hereditary cancer syndromes inform them of the possibility of discrimination and the legal protection that currently exists to protect against this.[4]

Conclusions

Genetic testing is a growing and expanding science. Guidelines to assist both oncology nurses and patients exist and position papers describing recommendations for surveillance are presently in place for some cancers.[5] Oncology nurses are in ideal positions to provide information about genetic testing as well as serve as patient advocates while promoting confidentiality regarding test results.[6]