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

  • Original Article
  • Published:
Journal of Gastrointestinal Surgery

Abstract

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.

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Authors and Affiliations

  1. Division of General Surgery, Department of Surgery, University of Utah Hospitals & Clinics, 50 N Medical Dr, Salt Lake City, UT, 84132, USA

    Austin R. Cannon & Thomas B. Pickron

  2. Huntsman Cancer Institute, Salt Lake City, USA

    Megan Keener & Deborah Neklason

  3. Division of Epidemiology, Department of Internal Medicine, University of Utah Hospitals & Clinics, Salt Lake City, USA

    Deborah Neklason

Authors
  1. Austin R. Cannon
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  2. Megan Keener
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  3. Deborah Neklason
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  4. Thomas B. Pickron
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Contributions

Authors Cannon, Neklason, and Pickron equally contributed to the conception and interpretation of this study. Author Keener was responsible for data acquisition, data fidelity, and assistance with data interpretation. Author Cannon was responsible for data organization and analysis. Author Cannon was responsible for creation of the initial written draft while authors Keener, Neklason, and Pickron were all equally involved in draft revisions and final submission.

Corresponding author

Correspondence to Austin R. Cannon.

Ethics declarations

All aspects of this study were approved by the University of Utah’s Institutional Review Board for human subject research.

Conflict of Interest

The authors declare that they have no conflicts of interest.

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Cannon, A.R., Keener, M., Neklason, D. et al. Surgical Interventions, Malignancies, and Causes of Death in a FAP Patient Registry. J Gastrointest Surg 25, 452–456 (2021). https://doi.org/10.1007/s11605-019-04412-9

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  • DOI: https://doi.org/10.1007/s11605-019-04412-9

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