High grade dysplasia in fundic gland polyps in a child with attenuated familial adenomatous polyposis and familial gastric cancer

Familial adenomatous polyposis (FAP) is a rare hereditary syndrome characterized by multiple colorectal polyps and early development of colorectal cancer. Although FAP uniformly involves the large bowel, it may also produce lesions in the stomach and upper intestinal tract. Fundic gland polyps are the most common gastric lesion in FAP. In the general population, these polyps are considered benign and have no malignant potential. However, in FAP patients, fundic gland polyps have been occasionally recognized as precursor lesions from which invasive cancer may develop. Herein, we present a case of gastric adenocarcinoma arising from fundic gland polyps in an FAP patient. We also review reported cases of gastric cancer in FAP and FAP variant patients in an effort to better understand the pathology, clinical course, and optimal screening and treatment strategies for this disease manifestation.

Download Full-text

Mo1182 Endoscopic Polypectomy Surveillance (EPS) Sampling of Fundic Gland Polyps in Familial Adenomatous Polyposis (FAP) Syndrome

Gastrointestinal Endoscopy ◽

10.1016/j.gie.2017.03.1053 ◽

2017 ◽

Vol 85 (5) ◽

pp. AB453

Author(s):

Jong Yeul Lee ◽

Laith H. Jamil ◽

Simon K. Lo

Keyword(s):

Familial Adenomatous Polyposis ◽

Endoscopic Polypectomy ◽

Fundic Gland ◽

Adenomatous Polyposis ◽

Fundic Gland Polyps

Download Full-text

Morphology and natural history of familial adenomatous polyposis-associated dysplastic fundic gland polyps

Histopathology ◽

10.1111/his.12393 ◽

2014 ◽

Vol 65 (3) ◽

pp. 353-362 ◽

Cited By ~ 32

Author(s):

Thomas Arnason ◽

Wen-Yih Liang ◽

Eduardo Alfaro ◽

Paul Kelly ◽

Daniel C Chung ◽

...

Keyword(s):

Familial Adenomatous Polyposis ◽

Natural History ◽

Fundic Gland ◽

Adenomatous Polyposis ◽

Fundic Gland Polyps ◽

History Of

Download Full-text

Appropriate Management of Attenuated Familial Adenomatous Polyposis: Report of a Case and Review of the Literature

Digestive Diseases ◽

10.1159/000497207 ◽

2019 ◽

Vol 37 (5) ◽

pp. 400-405 ◽

Cited By ~ 2

Author(s):

Aleksandra Sokic-Milutinovic

Keyword(s):

Familial Adenomatous Polyposis ◽

Apc Gene ◽

Colorectal Carcinogenesis ◽

Endoscopic Polypectomy ◽

Fundic Gland ◽

Adenomatous Polyposis ◽

Attenuated Familial Adenomatous Polyposis ◽

Increased Risk ◽

Asymptomatic Individuals ◽

Immediate Surgery

Hereditary polyposis syndromes in which APC gene germline mutations can lead to colorectal carcinogenesis are familial adenomatous polyposis (FAP), attenuated FAP (AFAP) and MUTYH-associated polyposis. All 3 syndromes increase the potential for the development of colorectal cancer. AFAP is diagnosed if less than 100 adenomas are detected in the colon at presentation. AFAP is inherited in an autosomal dominant manner. We present a case of a 22-year-old female with AFAP who was treated with endoscopic polypectomy and surveilled by annual colonoscopy. Guidelines for AFAP surveillance suggest annual colonoscopy with endoscopic polypectomy in asymptomatic individuals. Indications for immediate surgery include documented or suspected cancer or significant symptoms. Preferred surgical option in AFAP is colectomy and ileo-rectal anastomosis. Surveillance of the AFAP patients should include upper GI endoscopy and duodenoscopy with random biopsies of fundic gland polyps and endoscopic resection of detected adenomas. Annual thyroid ultrasound is indicated due to increased risk for thyroid cancer. In pediatric patients tested positive for germline mutation of APC gene screening for hepatoblastoma using alpha-fetoprotein and liver ultrasound should be performed.

Download Full-text