Table 15.

Statements pertaining to the upper gastrointestinal tract in familial adenomatous polyposis—extended version

Statements
Risk factors for upper GI neoplasia in FAP
UGI.1 What are the risk factors of developing duodenal adenocarcinoma? The risk factors most strongly associated with duodenal adenocarcinoma include Spigelman stage IV (either at first endoscopy or during surveillance), high-grade dysplasia in duodenal adenomas, duodenal adenomas >10 mm in diameter, and ageing. Additional risk factors have provided inconsistent evidence and need further evaluation.
UGI.2 What are the risk factors of developing adenocarcinoma of the papilla? A: The risk of papillary adenocarcinoma could increase with age. B: 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 (larger than 1 cm, high-grade dysplasia 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.  C: The Spigelman classification could underestimate the risk of developing a papillary adenocarcinoma.  D: Among the known pathogenic adenomatous polyposis coli gene variants, none have been identified as a risk factor for the development of papillary adenocarcinoma.
Surveillance
UGI.3 From what age should endoscopic surveillance of the upper GI be performed? Endoscopic surveillance of the upper gastrointestinal tract may start after the age of 18 years but no later than 30 years.
UGI.4 What is the optimal interval of endoscopic surveillance of the upper GI tract? A: 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. B: Duodenal surveillance intervals should be based on the Spigelman stage and the appearance of the papilla. Surveillance recommendations are illustrated in Fig. 4. C: Gastric surveillance intervals should depend on the number, the dimensions, and the histological characteristics of adenomas. Surveillance recommendations are illustrated in Fig. 5. D: Post-duodenal surgery surveillance intervals depend on the type of duodenal surgery performed. Surveillance recommendations are reported in Fig. 4.
UGI.5 What are the optimal modalities of endoscopic surveillance of the duodenum and the papilla? A: 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. B: Chromoendoscopy, both digital and dye-chromoendoscopy, could 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. C: Video-capsule endoscopy is not adequate for gastric, duodenal and papillary surveillance. D: 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.
UGI.6: No statement can be provided on the use of random duodenal biopsies.
UGI.7 Do we need random biopsies of polyps in the duodenum for endoscopic surveillance? A: 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. B: Taking random biopsies of the papilla improves the diagnosis of low-grade dysplasia. The benefit of random biopsies in macroscopically normal tissue to detect a high-grade dysplasia or an invasive adenocarcinoma of the papilla is very low, at least lower than 1% but not nil.
Spigelman staging system
UGI.8 Are current Spigelman staging-based surveillance and management recommendations optimal for prevention of duodenal cancer in FAP? 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.
UGI.9 What is the duodenal cancer risk for each Spigelman stage? A: 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. B: The estimated lifetime risk of duodenal cancer may be lowered after endoscopic or surgical downstaging.
Endoscopic treatment option
UGI.10 When should endoscopic resection be considered? A: 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 could be done. B: All non-papillary duodenal lesions >10 mm should undergo endoscopic resection C: Non-papillary duodenal lesions measuring 5–10 mm in size could undergo either endoscopic resection or surveillance. D: All papillary adenomas should be candidates for endoscopic resection, but especially if harbouring high-grade dysplasia, villous histology, or if >10 mm in size. E: All gastric adenomas larger than 5 mm should undergo endoscopic resection. F: 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.
Duodenal surgery versus endoscopic management
UGI.11 When should surgical resection be considered? A: Curative surgical resection must be offered to surgically resectable, histologically proven duodenal and ampullary adenocarcinoma. B: Prophylactic surgical resection could be considered for Spigelman stage IV duodenal polyposis. C: Prophylactic surgical resection could be considered for Spigelman stage II–III that is not endoscopically manageable. D: Papillary adenomas >10 mm or with high-grade dysplasia should undergo endoscopic resection, rather than surgical resection, if feasible.
UGI.12 When should endoscopic versus surgical resection be considered? A: All duodenal, papillary and gastric lesions with histologically proven invasive carcinoma should undergo surgery (if surgically completely resectable). B: Spigelman stage 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. C: Papillary and duodenal adenomas should undergo endoscopic resection, rather than surgery, if feasible.
UGI.13 Should pancreas-sparing duodenectomy or pancreatico-duodenectomy be preferred when prophylactic surgery is required? 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.
Management of gastric findings
UGI.14 Is treatment for fundic gland polyposis indicated? A: Endoscopic resection of fundic gland polyps has not been demonstrated to reduce the risk of gastric adenocarcinoma. However, in cases of large or symptomatic fundic gland polyps, endoscopic resection may be considered after expert evaluation. B: Fundic gland polyposis may progress to gastric adenocarcinoma in patients with FAP. Such risk cannot be quantified up to now.
UGI.15 What treatment modalities are available for fundic gland polyposis? Endoscopic resection may be a consideration for fundic gland polyps that are large or symptomatic, after expert evaluation.
UGI.16 Is treatment for gastric adenoma indicated? Suspected gastric adenomas should be removed, endoscopically if feasible.
Management of small intestinal findings including post upper GI surgery
UGI.17 Is endoscopic surveillance of the neo-duodenum and jejunum indicated after surgery? After surgery, the neo-duodenum and jejunum should receive endoscopic surveillance.
UGI.18 Is surveillance of the small bowel indicated? Small bowel surveillance is not routinely indicated, but small bowel examination is recommended before duodenal surgical intervention.
UGI.19 What modality should be preferred for small bowel surveillance: video-capsule endoscopy, single-/double-balloon enteroscopy, or small bowel MRI? 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.

FAP, familial adenomatous polyposis; GI, gastrointestinal.