. 2024 May 9;111(5):znae070. doi: 10.1093/bjs/znae070

Table 2.

Short version: statements pertaining to upper gastrointestinal manifestations in familial adenomatous polyposis

Statements	Level of evidence and agreement
Risk factors for upper GI neoplasia in FAP
UGI.1: The risk factors most strongly associated with duodenal adenocarcinoma include Spigelman stage IV (either at first endoscopy or during surveillance), HGD in duodenal adenomas, duodenal adenomas >10 mm in diameter, and ageing. Additional risk factors have provided inconsistent evidence and need further evaluation.	LE: low Agreement: 93% (SA 55%; A 38%; N 3%; D 4%)
UGI.2A: The risk of papillary adenocarcinoma could increase with age.	LE: Very low Agreement: 89% (SA 43%; A 46%; N 6%; D 5%)
UGI.2B: Papillary adenoma could be a risk factor of papillary adenocarcinoma. A personal history of extra-intestinal manifestations could increase the risk of developing a papillary adenoma. An advanced papillary adenoma (>1 cm, HGD and villous or tubular-villous component) increases the papillary carcinoma risk. The association between male gender, a personal history of cholecystectomy and/or a personal history of extra-colonic malignancy as risk factors of papillary carcinoma is uncertain and needs further investigation.	LE: Very low Agreement: 90% (SA 38%; A 52%; N 3%; D 7%)
UGI.2C: The Spigelman classification could underestimate the risk of developing a papillary adenocarcinoma.	LE: Low Agreement: 90% (SA 38%; A 52%; N 3%; D 7%)
UGI.2D: Among the known pathogenic adenomatous polyposis coli gene variants, none have been identified as a risk factor for the development of papillary adenocarcinoma.	LE: Very low Agreement: 100% (SA 29%; A 71%)
Surveillance
UGI.3: Endoscopic surveillance of the upper GI tract may start after the age of 18 years but no later than 30 years.	LE: Low Agreement: 89% (SA 35%; A 54%; N 4%; D 7%)
UGI.4A: Surveillance intervals depend on gastric, duodenal and neo-duodenal (post-surgical) endoscopic findings. The site with the most advanced stage should direct the surveillance interval.	LE: Low Agreement: 89% (SA 71%; A 18%; N 11%)
UGI.4B: Duodenal surveillance intervals should be based on the Spigelman stage and the appearance of the papilla. Surveillance recommendations are illustrated in Fig. 4.	LE: Low Agreement: 89% (SA 50%; A 39%; N 7%; D 4%)
UGI.4C: Gastric surveillance intervals should depend on the number, the dimensions and the histological characteristics of adenomas. Surveillance recommendations are illustrated in Fig. 5.	LE: Low Agreement: 89% (SA 45%; A 44%; N 7%; D 4%)
UGI.4D: Post-duodenal surgery surveillance intervals depend on the type of duodenal surgery performed. Surveillance recommendations are reported in Fig. 4.	LE: Low Agreement: 82% (SA 43%; A 39%; N 11%; D 4%; SD 3%)
UGI.5A: Duodenal and papillary surveillance could rely on cap-assisted forward-viewing endoscopy for complete visualization of the papilla. If the papilla is not adequately viewed, side-viewing endoscopy should be used.	LE: Moderate Agreement: 92% (SA 65%; A 27%; N 4%; D 4%)
UGI.5B: Chromoendoscopy, both digital and dye-chromoendoscopy, can be used to improve the visualization of duodenal, papillary and gastric adenomas. Narrow-band imaging could also improve the visualization of duodenal and papillary adenomas.	LE: Moderate Agreement: 81% (SA 39%; A 42%; N 15%; D 4%)
UGI.5C: Video-capsule endoscopy is not adequate for gastric, duodenal and papillary surveillance.	LE: Low Agreement: 96% (SA 65%; A 31%; N 4%)
UGI.5D: Endoscopic ultrasound and double-balloon enteroscopy are not part of routine endoscopic surveillance, but they could be useful as second-level diagnostic and/or therapeutic exams.	LE: Low Agreement: 85% (SA 54%; A 31%; N 15%)
UGI.6: No statement can be provided on the use of random duodenal biopsies.	LE: — Agreement: 87% (SA 54%; A 33%; N 13%)
UGI.7A: The impact of random biopsies on the prevention of papillary adenocarcinoma is unknown. Thus, no formal recommendations to adopt or not this strategy of systematic random papillary biopsies can be made.	LE: Very low Agreement: 86% (SA 38%; A 48%; N 14%)
UGI.7B: Taking random biopsies of the papilla improves the diagnosis of low-grade dysplasia. The benefit of random biopsies in macroscopically normal tissue to detect an HGD or an invasive adenocarcinoma of the papilla is very low, at least lower than 1% but not nil.	LE: High Agreement: 86% (SA 52%; A 34%; N 10%; D 4%)
Spigelman staging system
UGI.8: The Spigelman stage-based management provides the highest available level of evidence for duodenal cancer prevention. However, there are limitations to the Spigelman stage, which could be improved upon.	LE: Low Agreement: 97% (SA 59%; A 38%; N 3%)
UGI.9A: The average lifetime risk of duodenal cancer is estimated to be up to 30% for Sp-IV, 13% for Sp-III, 12% for Sp-II, and lower than 5% for Sp-I and Sp-0.	LE: Low Agreement: 89% (SA 22%; A 67%; N 11%)
UGI.9B: The estimated lifetime risk of duodenal cancer may be lowered after endoscopic or surgical downstaging.	LE: Low Agreement: 100% (SA 35%; A 65%)
Endoscopic treatment option:
UGI.10A: Endoscopic downstaging should be personalized according to endoscopic findings. Ideally, Spigelman stage IV should be downstaged as much as possible. An attempt to downstage Spigelman stage III can be performed.	LE: Low Agreement: 85% (SA 31%; A 54%; N 8%; D 4%; SD 3%)
UGI.10B: All non-papillary duodenal lesions >10 mm should undergo endoscopic resection.	LE: Moderate Agreement: 93% (SA 52%; A 41%; D 7%)
UGI.10C: Non-papillary duodenal lesions measuring 5–10 mm in size could undergo either endoscopic resection or surveillance.	LE: Low Agreement: 82% (SA 21%; A 61%; N 11%; D 7%)
UGI.10D: All papillary adenomas should be candidates for endoscopic resection, but especially if harbouring HGD, villous histology, or if >10 mm in size.	LE: Low Agreement: 85% (SA 52%; A 33%; N 7%; D 8%)
UGI.10E: All gastric adenomas larger >5 mm should undergo endoscopic resection.	LE: Low Agreement: 83% (SA 28%; A 55%; N 10%; D 7%)
UGI.10F: All gastric, duodenal and ampullary histologically proven carcinomas with endoscopic features suggestive of invasive adenocarcinoma should undergo surgery with or without systemic therapy, rather than endoscopic resection.	LE: Strong Agreement: 93% (SA 72%; A 21%; D 7%)
*Duodenal surgery versus* endoscopic management**
UGI.11A: Curative surgical resection must be offered to surgically resectable, histologically proven duodenal and ampullary adenocarcinoma.	LE: Strong Agreement: 100% (SA 86%; A 14%)
UGI.11B: Prophylactic surgical resection could be considered for Spigelman stage IV duodenal polyposis.	LE: Moderate Agreement: 88% (SA 31%; A 57%; N 8%; D 4%)
UGI.11C: Prophylactic surgical resection could be considered for Spigelman stage II–III that is not endoscopically manageable.	LE: Low Agreement: 88% (SA 31%; A 57%; N 8%; D 4%)
UGI.11D: Papillary adenomas >10 mm or with HGD should undergo endoscopic resection, rather than surgical resection, if feasible.	LE: Low Agreement: 86% (SA 54%; A 32%; N 14%)
UGI.12A: All duodenal, papillary and gastric lesions with histologically proven invasive carcinoma should undergo surgery (if surgically completely resectable).	LE: Strong Agreement: 100% (SA 86%; A 14%)
UGI.12B: Spigelman stages III and IV duodenal polyposis without evidence of invasive tumour should undergo endoscopic treatment, if feasible, rather than surgical resection. However, there should be a low threshold to offer surgical resection once downstaging appears no longer manageable endoscopically.	LE: Low Agreement: 93% (SA 50%; A 43%; N 7%)
UGI.12C: Papillary and duodenal adenomas should undergo endoscopic resection, rather than surgery, if feasible.	LE: Low Agreement: 96% (SA 48%; A 48%; N 4%)
UGI.13: Pancreato-duodenectomy is the procedure of choice in case of suspected duodenal cancer. For prophylactic surgery, both pancreas-sparing duodenectomy and pancreatico-duodenectomy may be considered.	LE: Low Agreement: 100% (SA 35%; A 65%)
Management of gastric findings
UGI.14A: Endoscopic resection of FGPs has not been demonstrated to reduce the risk of gastric adenocarcinoma. However, in cases of large or symptomatic FGPs, endoscopic resection may be considered after expert evaluation.	LE: Low Agreement: 100% (SA 35%; A 65%)
UGI.14B: Fundic gland polyposis may progress to gastric adenocarcinoma in patients with FAP. Such risk cannot be quantified up to now.	LE: Very low Agreement: 82% (SA 18%; A 64%; N 7%; D 11%)
UGI.15: Endoscopic resection may be a consideration for FGPs that are large or symptomatic, after expert evaluation.	LE: Low Agreement: 93% (SA 39%; A 54%; N 7%)
UGI.16: Suspected gastric adenomas should be removed, endoscopically if feasible.	LE: Low Agreement: 100% (SA 56%; A 44%)
Management of small intestinal findings including post UGI surgery
UGI.17: After surgery, the neo-duodenum and jejunum should receive endoscopic surveillance.	LE: Low Agreement: 100% (SA 75%; A 25%)
UGI.18: Small bowel surveillance is not routinely indicated, but small bowel examination is recommended before duodenal surgical intervention.	LE: Low Agreement: 100% (SA 42%; A 58%)
UGI.19: When examination of the small bowel is indicated, video-capsule endoscopy is the method of choice. If positive, patients should undergo enteroscopy for diagnosis and therapy.	LE: Low Agreement: 95% (SA 16%; A 79%; D 5%)

A, agree; D, disagree; FAP, familial adenomatous polyposis; FGP, fundic gland polyp; GI, gastrointestinal; HGD, high-grade dysplasia; LE, level of evidence; N, neutral; SA, strongly agree; SD, strongly disagree.