FAP with Duodenal Adenomas: Surgical Management When Endoscopic Downstaging Has Not Reached Target Spigelman Stage

Patients with familial adenomatous polyposis (FAP) frequently develop upper GI lesions — gastric adenomas, duodenal adenomas, and papillary (ampullary) adenomas — alongside duodenal polyposis. This protocol addresses the surgical step that becomes appropriate when endoscopic management has reached the limit of what it can achieve.

When the previous step was not sufficient

Endoscopic resection and endoscopic downstaging of duodenal polyposis are the initial approach for upper GI lesions in FAP. When downstaging does not successfully reduce duodenal polyposis to Spigelman stage 0–III, or when endoscopic features suggest invasive disease requiring more definitive management, the protocol moves to a surgical pathway.

Surgical approach — partial overview

The protocol covers duodenal surgery as the next step, with options that vary depending on whether the indication is prophylactic or driven by histologically confirmed invasive carcinoma. Which specific procedure applies — and under what conditions — is detailed in the full protocol.

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References

DOI: 10.1093/bjs/znae263

All gastric adenomas larger >5 mm should undergo endoscopic resection.

All non-papillary duodenal lesions >10 mm should undergo endoscopic resection.

All papillary adenomas should be candidates for endoscopic resection, but especially if harbouring HGD, villous histology, or if >10 mm in size.

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.

Curative surgical resection must be offered to surgically resectable, histologically proven duodenal and ampullary adenocarcinoma.

Prophylactic surgical resection could be considered for Spigelman stage IV duodenal polyposis.

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.

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