Genetic cancers are relatively uncommon but not rare. Although there has not been a comprehensive study of the incidence of cancers that are caused by an identifiable single gene mutation, it is estimated that they account for approximately 5% to 10% of all cancers, or 50,000 to 100,000 patients annually in the U.S. 1 The hallmarks of a genetic cancer syndrome are early onset, multiple family members in multiple generations with cancer, bilateral cancer, and multiple cancers in the same person.
Until recently, the VA has not had a significant interest in genetic cancer risk assessment (GCRA). This is changing, however, because veterans with identified genetic risks for cancer can benefit from targeted screening and intervention strategies to lower their risk of dying of cancer. The value of GCRA was also recognized in the 2015 standards for accreditation of the American College of Surgeons, which include a requirement for programs to include a provision for GCRA. 2
The 2 most common familial cancer syndromes are hereditary breast and ovarian cancer (HBOC) syndrome, which occurs in about 5% of all patients with breast cancer, and Lynch syndrome (LS), or hereditary nonpolyposis colorectal cancer (CRC) syndrome, which occurs in about 3% of all patients with CRC. 3,4 Other familial cancer syndromes are rare: For example, familial adenomatous polyposis (FAP) accounts for 0.2% to 0.5% of all CRC cases. 5
The Raymond G. Murphy VAMC in Albuquerque is the sole VA hospital in New Mexico. Its catchment area extends into southern Colorado, eastern Arizona, and western Texas. About 40 CRCs and 8 breast cancers are diagnosed at this facility yearly. Given the incidence of these familial cancer syndromes, one might expect to see 1 LS case/year, 1 HBOC case every 2 years, and 1 FAP or attenuated FAP case every 5 to 10 years.
Methods
In 2010, a GCRA clinic was set up to evaluate and manage treatment of veterans who might have inherited a genetic cancer syndrome. Prior to that, veterans with suspected genetic cancer family syndromes were referred to the University of New Mexico for evaluation and testing. Initially, the pathology department (PD) paid for genetic testing. However, due to the cost of testing, a formal budget for genetic testing was approved. Contracts were set up by the PD with outside laboratories for genetic testing services. For quality control, all veterans who were referred for genetic evaluation were seen by Dr. Lin.
The initial consultation consisted of construction of a family pedigree and evaluation, using available models or tables, such as the Myriad tables (BRCA), Penn II BRCA, or PREMM 1,2,6 (LS), to estimate likelihood of finding a mutation. Veterans who had a 10% likelihood of finding a gene mutation were counseled, following the American Society of Clinical Oncology guidelines (Table 1). Those who consented to genetic testing signed a consent form and were given a copy of that form and a copy of their family pedigree. Because the VA covers the cost of counseling and testing, cost was not discussed.