Advanced duodenal neoplasia and carcinoma in familial adenomatous polyposis: outcomes of surgical management

Abstract

Background: In addition to the presence of neoplasia in the colon and rectum, patients with familial adenomatous polyposis (FAP) may develop numerous polyps and carcinoma within the upper gastrointestinal tract. Methods:The aim of the present paper was to review the incidence advanced duodenal polyposis or cancer and their surgical outcomes. A retrospective review of patients' records from our department was performed. respectively. Duodenal carcinomas occurred later (55.8 years) when compared to advanced adenomatosis (45.3 years). Three patients were diagnosed due to symptoms, while the others were detected under endoscopic surveillance. Age interval between FAP treatment and duodenal neoplasia diagnosis was 15.3 years . All but one patient underwent duodenopancreatectomy (DP). Two from the 7 patients undergoing DP died, one from pulmonary embolism 30 days after surgery and the other from recurrent T4N0 duodenal tumor. Thus, major operative morbidity and mortality were 12.5%. Conclusions:In this single-center Brazilian series of FAP patients: (I) advanced duodenal neoplasia or cancer requiring surgery occurred in 5.5% of patients; (II) when reaching the fifth decade of life, patients should be carefully evaluated to diagnose and treat early lesions; (III) in spite of the technical complexity of DP, operative morbidity is acceptable in experienced hands; and (IV) continuous surveillance is necessary during follow-up.

Endoscopic management of polyposis syndromes: European Society of Gastrointestinal Endoscopy (ESGE) Guideline

“…Two retrospective studies reported that 4 % -6 % of the FAP patients had been surgically treated for duodenal polyposis, describing mortality rates after pancreas-preserving duodenectomy ranging from 5 % to 33 % [105,106]. The in-hospital morbidity was 49 %, without differences between patients with benign adenomatosis and cancer [106].…”

Survival outcomes after surgical management of sporadic or familial adenomatous polyposis associated duodenal cancer

“…This is the only series that compares FAP related duodenal adenocarcinoma to sporadic duodenal adenocarcinoma (Table 5). 12,19‐28 A previous study which included patients that underwent palliative surgeries and those with metastatic disease with FAP‐associated duodenal cancer noted a median survival of 11 months 26 . While that is still probably true, we noted an overall median survival of 7.4 years in patients who underwent surgery with curative intent.…”

Ampullary cancer in a patient with familial adenomatous polyposis – a rare case report and current status of management

“…In fact, the surveillance should start after 25 years of age. When necessary, radical procedure such as PD may be performed with acceptable morbidity by skilled surgical teams (29).…”