Surgical Interventions, Malignancies, and Causes of Death in a FAP Patient Registry.

Background: Familial adenomatous polyposis (FAP) patients are at risk for numerous malignancies. Multiple surgeries exist to mitigate the risk of colorectal cancer. Surgeons must weigh future quality of life versus the risk of dysplasia. As FAP patient longevity increases, there remains a risk of other malignancies. This study examines surgical interventions, development of cancers, and causes of mortality in a FAP registry.

Methods: Patients with FAP or attenuated FAP (aFAP) were identified by linking the Hereditary Gastrointestinal Cancer Registry with University of Utah's medical records. Patients without sufficient information were excluded. Patient demographics, surgical histories, cancer diagnoses, and causes of death were extracted. Logrank and Fisher's exact tests were employed to detect significant differences between groups.

Results: After exclusion criteria, 140 patients were analyzed. Sixty patients (42.9%) underwent total proctocolectomy with ileal pouch-anal anastomosis (IPAA) followed by 50 (35.7%) having total colectomy with ileorectal anastomosis (IRA). IPAA patients were more likely female (p = 0.01) and have FAP (p < 0.01) versus IRA patients. Nineteen patients (15.0%) required additional colorectal surgeries; however, no differences were based on initial surgery. Colorectal cancer was diagnosed in 22 patients (15.7%), while 7 (5.0%) developed gastric cancer. Of the 15 deceased patients, 6 (40%) died due to gastric adenocarcinoma.

Discussion: This study suggests that aFAP and FAP patients are undergoing appropriate colorectal interventions to reduce colorectal cancer mortality; however, repeat interventions are frequent. Gastric malignancy is common and represents the leading cause of death. Further studies are needed to determine appropriate surveillance protocols to reduce this risk of malignancy.