Familial adenomatous polyposis

Alarcon, Fabricio J.; Burke, Carol A.; Church, James M.; Stolk, Rosalind U. van

doi:10.1007/bf02236201

Diseases of the Colon &Amp; Rectum

1999

DOI: 10.1007/bf02236201

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Familial adenomatous polyposis

Fabricio J. Alarcon¹,

Carol A. Burke²,

James M. Church

et al.

Abstract: Endoscopic eradication is an appropriate initial treatment for histologically advanced, noncancerous neoplasms or for patients who are not surgical candidates. Pancreas-sparing duodenectomy may be the treatment of choice for patients with carcinoma and those who have failed endoscopic therapy.

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Cited by 64 publications

(2 citation statements)

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“…In patients with familial adenomatous polyposis (FAP) the management of duodenal and ampullary lesions is particularly controversial because of local recurrence. Some have suggested that repeated endoscopic therapy is a reasonable option for histologically advanced, non-cancerous lesions [7], whereas others have recommended early radical surgery [8]. The relative roles of endoscopy, local resection, and radical resection in the management of peri-ampullary lesions for FAP is still not clearly defined [9], and peri-ampullary carcinoma is now the leading cause of death in patients afflicted with this syndrome.…”

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confidence: 99%

Transduodenal Resection of Peri‐Ampullary Lesions

et al. 2005

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Transduodenal resection (TDR) of lesions near the ampulla of Vater is an alternative to the Whipple pancreaticoduodenectomy. A retrospective analysis was performed to determine the long-term outcome and the utility of intraoperative frozen section examinations in aiding operative decision making in patients undergoing TDR. From 1992 to 2002, 19 patients with an average age of 64.2 years (range: 33-84 years) underwent a transduodenal resection of a peri-ampullary lesion; median follow-up was 47 months (range: 2-100 months). Pathology of the lesions was as follows: 11 with benign ampullary adenomas, including 4 with familial adenomatous polyposis (FAP); 7 with peri-ampullary adenocarcinomas; and 1 with a benign stricture. Survival for the entire cohort is 100%. In 12 cases an intraoperative frozen section was performed. The specificity and positive predictive value of the intraoperative histology were both 100%, and the sensitivity and negative predictive value were 57% and 38%, respectively. Three of the 4 patients with FAP have recurrent adenomatous change; 2 of the 7 with carcinoma have metastatic adenocarcinoma. Transduodenal resection of peri-ampullary lesions appears to be a safe alternative to radical resection for benign adenomas and selected carcinoma. Intraoperative frozen section assessment is recommended in cases of potential adenocarcinoma.

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Transduodenal Resection of Peri‐Ampullary Lesions

et al. 2005

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“…However, it is also noted that nearly 50% of patients with adenocarcinoma have not had Spigelman stage IV disease 5 . Guidelines suggest patients with advanced duodenal polyposis be considered for prophylactic duodenectomy including pancreaticoduodenectomy (PD), pancreas-sparing duodenectomy (PSD), or segmental duodenectomy (SD), which is more effective than local surgical or endoscopic polypectomy 6–8 …”

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The Prevalence and Significance of Jejunal and Duodenal Bulb Polyposis After Duodenectomy in Familial Adenomatous Polyposis

et al. 2019

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Objective: To evaluate the prevalence, natural history, and severity of polyposis of the duodenal bulb and jejunum after duodenectomy in patients with FAP. Summary of Background Data: Advanced duodenal polyposis stage in FAP requires consideration of duodenal resection to prevent cancer; pyloruspreserving approach of pancreas-sparing duodenectomy (PSD) is preferred. Post-duodenectomy data indicate polyps occur in the duodenal bulb and the post-anastomotic jejunum, but limited data exists regarding their significance. Methods: We identified consecutive FAP patients After duodenal resection, including pancreaticoduodenectomy, PSD, or segmental duodenectomy, at Cleveland Clinic. Medical records were used to determine time to diagnosis of duodenal bulb or jejunal polyps, length of follow up, and severity of polyposis including maximal Spigelman stage (SS) of jejunal polyposis (neo-SS). Results: 64 patients with FAP underwent duodenectomy and endoscopic follow up. 28% underwent pancreaticoduodenectomy, 61% PSD, and 11% segmental duodenectomy. Postoperatively, 38/64 (59%) were diagnosed with jejunal polyposis, with median time to diagnosis of 55 months and follow up time of 127 months. Jejunal polyposis was advanced in 21% (neo-SS III or IV). Fifty percent were treated endoscopically, 1 patient required surgery. Jejunal polyp-free survival after duodenectomy differed by surgery type (P ¼ 0.008). A total of 55/64 patients underwent a pylorus-preserving procedure, and 6/55 (11%) developed duodenal bulb polyps. All bulb polyps were large (>20 mm) and found after PSD. Endoscopic resection was unsuccessful in 5 patients, but no surgical intervention was required. Conclusions: Polyposis occurs in the remaining duodenal and jejunal mucosa in the majority of patients after surgical duodenectomy. Jejunal polyposis is advanced in 1 in 5 patients, but rarely requires surgery. Endoscopic management of jejunal polyposis seems feasible but has proven difficult for duodenal bulb polyps.

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Worldwide survey among polyposis registries of surgical management of severe duodenal adenomatosis in familial adenomatous polyposis

Cappel

Järvinen

Björk

et al. 2003

British Journal of Surgery

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Familial adenomatous polyposis

Cited by 64 publications

References 16 publications

Transduodenal Resection of Peri‐Ampullary Lesions

Transduodenal Resection of Peri‐Ampullary Lesions

The Prevalence and Significance of Jejunal and Duodenal Bulb Polyposis After Duodenectomy in Familial Adenomatous Polyposis

Worldwide survey among polyposis registries of surgical management of severe duodenal adenomatosis in familial adenomatous polyposis

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