A fourteen-year follow-up of a familial adenomatous polyposis case complicated with desmoid tumours: Comments and computed tomography evaluation

Abstract Background and study aims Restorative proctocolectomy has become the most common surgical option for patients with familial adenomatous polyposis (FAP). However, adenomas and even carcinomas may develop in the ileal pouch over time. The aim of this study was to evaluate the long-term incidence and nature of ileal pouch or distal ileal adenomas and carcinomas in patients with FAP. Patients and methods This was a retrospective study of 47 FAP patients with Kock’s continent ileostomy (Kock) (n = 8), ileorectal anastomosis (IRA) (n = 13), and ileal pouch-anal anastomosis (IPAA) (n = 26). Patients were followed with a standardized protocol including chromoendoscopy and biopsies of visible polyps in the ileal pouch, distal ileum, and rectum every 6 to 12 months. Results Median follow-up was 21.0 years. Overall risk of adenoma development was significantly higher in IRA patients, with incidence rates of 85 % and 100 % at 5 and 10 years’ follow-up, respectively, compared to pouch patients (Kock + IPAA) (P < 0.001). However, there was also a high frequency of adenomas in the ileal pouch mucosa, with rates of 12 %, 33 %, and 68 %, at 5, 10, and 20 years of follow-up, respectively. Maximum size of ileal pouch adenomas was significantly related to time since surgery (P = 0.0214). Six cases of advanced adenomas including two cases of adenocarcinomas developed in the ileal pouch mucosa. Conclusions There is a significant incidence of adenoma(s) in the ileal pouch of FAP patients on long-term follow-up. Regular endoscopic surveillance is recommended, not only in IRA patients, but also in pouch patients after proctocolectomy.

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Long-term clinical outcomes and follow-up status in Japanese patients with familial adenomatous polyposis after radical surgery: a descriptive, retrospective cohort study from a single institute

International Journal of Colorectal Disease ◽

10.1007/s00384-020-03524-y ◽

2020 ◽

Vol 35 (4) ◽

pp. 675-684 ◽

Cited By ~ 1

Author(s):

Akihito Babaya ◽

Tomoki Yamano ◽

Takaaki Matsubara ◽

Yuya Takenaka ◽

Jihyung Song ◽

...

Keyword(s):

Cohort Study ◽

Familial Adenomatous Polyposis ◽

Clinical Outcomes ◽

Retrospective Cohort Study ◽

Retrospective Cohort ◽

Radical Surgery ◽

Japanese Patients ◽

Adenomatous Polyposis

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Follow-up Study of Associated Tumors in Cases of Familial Adenomatous Polyposis

Hereditary Colorectal Cancer ◽

10.1007/978-4-431-68337-7_12 ◽

1990 ◽

pp. 81-89

Author(s):

Kyosuke Ushio

Keyword(s):

Familial Adenomatous Polyposis ◽

Adenomatous Polyposis ◽

Follow Up Study ◽

Associated Tumors

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Gastric adenomas and their management in familial adenomatous polyposis

Endoscopy ◽

10.1055/a-1265-2716 ◽

2020 ◽

Author(s):

Isabel Martin ◽

Victorine H. Roos ◽

Chukwuemeka Anele ◽

Sarah-Jane Walton ◽

Victoria Cuthill ◽

...

Keyword(s):

Gastric Cancer ◽

Familial Adenomatous Polyposis ◽

Initial Diagnosis ◽

Gastric Adenoma ◽

Adenomatous Polyposis ◽

Recurrence Rates ◽

Patient Demographics ◽

Increased Risk ◽

Piecemeal Resection

Abstract Background Patients with familial adenomatous polyposis (FAP) are at increased risk of developing gastric adenomas. There is limited understanding of their clinical course and no consensus on management. We reviewed the management of gastric adenomas in patients with FAP from two centers. Methods Patients with FAP and histologically confirmed gastric adenomas were identified between 1997 and 2018. Patient demographics, adenoma characteristics, and management/surveillance outcomes were collected. Results Of 726 patients with FAP, 104 (14 %; 49 female) were diagnosed with gastric adenomas at a median age of 47 years (range 19 – 80). The median size of gastric adenomas was 6 mm (range 1.5 – 50); 64 (62 %) patients had adenomas located distally to the incisura. Five patients (5 %) had gastric adenomas demonstrating high-grade dysplasia (HGD) on initial diagnosis, distributed equally within the stomach. The risk of HGD was associated with adenoma size (P = 0.04). Of adenomas > 20 mm, 33 % contained HGD. Two patients had gastric cancer at initial gastric adenoma diagnosis. A total of 63 patients (61 %) underwent endoscopic therapy for gastric adenomas. Complications occurred in three patients (5 %) and two (3 %) had recurrence, all following piecemeal resection of large (30 – 50 mm) lesions. Three patients were diagnosed with gastric cancer at median follow-up of 66 months (range 66 – 115) after initial diagnosis. Conclusions We observed gastric adenomas in 14 % of patients with FAP. Of these, 5 % contained HGD; risk of HGD correlated with adenoma size. Endoscopic resection was feasible, with few complications and low recurrence rates, but did not completely eliminate the cancer risk.

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