Gastric tumours in FAP
Sarah-Jane Walton1,2, Ian M Frayling3, Susan K Clark4,5
1The Polyposis Registry, St Mark's Hospital, Watford Road, Harrow, London, UK. sarah-janewalton@nhs.net.
Familial Cancer
|March 9, 2017
Summary
Gastric cancer is uncommon in Familial Adenomatous Polyposis (FAP) in Western countries, often presenting late with poor outcomes. A link between gastric tumors and desmoid disease may exist.
Area of Science:
- Gastroenterology
- Oncology
- Genetics
Background:
- Familial Adenomatous Polyposis (FAP) is a hereditary condition predisposing individuals to numerous gastrointestinal polyps.
- Gastric cancer is not typically recognized as a primary extra-colonic manifestation of FAP, with limited data on gastric adenomas in this population.
Purpose of the Study:
- To investigate the clinical characteristics of gastric tumors (adenomas and carcinomas) in patients with FAP.
- To determine the prevalence, stage, management, and survival associated with gastric tumors in a European FAP cohort.
Main Methods:
- Retrospective analysis of patients diagnosed with gastric adenoma or adenocarcinoma within the largest European polyposis registry.
- Data collected included patient demographics, APC mutation status, tumor characteristics, surveillance history, and outcomes.
Main Results:
- Eight patients developed gastric cancer and 21 developed gastric adenomas.
- Gastric cancers presented at advanced stages (T3/4) with nodal or metastatic spread in most cases, leading to poor prognosis (median survival 13.5 months).
- An association was noted between gastric tumors and desmoid development (present in 88% of gastric cancer cases and 52% of adenoma cases).
Conclusions:
- Gastric cancer appears to be an infrequent extra-colonic manifestation of FAP in the Western world.
- Gastric tumors in FAP patients often present late and have a poor prognosis.
- Further multicenter studies are needed to confirm the association between gastric tumors and desmoid occurrence in FAP.
