Gastric tumours in FAP

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Title: Gastric tumours in FAP
Author(s): Walton, SJ
Frayling, IM
Clark, SK
Latchford, A
Item Type: Journal Article
Abstract: Gastric cancer is not a recognised extra-colonic manifestation of FAP, except in countries with a high prevalence of gastric cancer. Data regarding gastric adenomas in FAP are sparse. The aim of this study was to review the clinical characteristics of gastric tumours occurring within an FAP population from the largest European polyposis registry. All patients that developed a gastric adenoma or carcinoma were identified from a prospectively maintained registry database. The primary outcome measure was the occurrence of gastric adenoma or adenocarcinoma. Secondary outcomes included APC mutation, tumour stage, management and survival. Eight patients developed gastric cancer and 21 an adenoma (median age 52 and 44 years, respectively). Regular oesophagogastroduodenoscopy surveillance was performed in 6/8 patients who developed cancer. Half were advanced T3/4 tumours and 6/8 had nodal or metastatic spread at diagnosis. All cancer cases died within a median of 13.5 months from diagnosis. Gastric adenomas were evenly distributed: 11/21 (52%) in the distal and 10/21 (48%) proximal stomach, whereas 5/8 (63%) cancers were located proximally. An association between gastric tumour and desmoid development was observed; 7/8 (88%) cancer and 11/21 (52%) adenoma cases had a personal or family history of desmoid. It would appear from this small, retrospective study that gastric cancer is not a prominent extra-colonic feature of FAP in the Western world. It seems to present at an advanced stage with a poor prognosis. There may be an association between gastric tumour and desmoid occurrence but a large multicentre cohort is necessary to investigate this further.
Publication Date: 7-Mar-2017
Date of Acceptance: 1-Mar-2017
URI: http://hdl.handle.net/10044/1/45900
DOI: https://dx.doi.org/10.1007/s10689-017-9966-0
ISSN: 1573-7292
Publisher: Springer Verlag (Germany)
Start Page: 363
End Page: 369
Journal / Book Title: Familial Cancer
Volume: 16
Issue: 3
Copyright Statement: © 2017 Springer Science+Business Media Dordrecht. The final publication is available at Springer via https://dx.doi.org/10.1007/s10689-017-9966-0
Keywords: Science & Technology
Life Sciences & Biomedicine
Oncology
Genetics & Heredity
Gastric cancer
Gastric adenoma
Desmoid
Familial adenomatous polyposis
FAMILIAL ADENOMATOUS POLYPOSIS
UPPER GASTROINTESTINAL CANCER
SOMATIC MUTATION
DUODENAL POLYPS
RISK-FACTORS
GASTRECTOMY
PREVALENCE
MORTALITY
GERMLINE
GENOTYPE
Desmoid
Familial adenomatous polyposis
Gastric adenoma
Gastric cancer
1112 Oncology And Carcinogenesis
Oncology & Carcinogenesis
Publication Status: Published
Appears in Collections:Division of Surgery
Faculty of Medicine



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