Pancreas-sparing total duodenectomy for Spigelman stage IV duodenal polyposis associated with familial adenomatous polyposis: experience of 10 cases at a single institution

Abstract

Duodenal cancer is a leading cause of death in patients with familial adenomatous polyposis (FAP). In patients with Spigelman's classification (SC) stage IV duodenal polyposis (DP), careful endoscopic surveillance by specialists or surgical intervention is mandatory. We herein report the surgical and pathological outcomes of FAP patients with SC stage duodenal polyposis undergoing pancreas-sparing total duodenectomy (PSTD), which has been rarely reported but seems optimal in such patients. PSTD and distal gastrectomy with Billroth-I type reconstruction in ten consecutive FAP patients with SC stage IV DP are reported. The median duration of surgery was 396 min (range 314-571 min) and the median estimated blood loss was 480 mL (range 100-975 mL). Significant postoperative complications included wound infection in 1 patient, pancreatic fistula [International Study Group on Pancreatic Fistula definition (ISGPF) grade B] in 4 patients. Histopathologic examinations revealed a well-differentiated carcinoma in situ in 3 patients and others were all adenomas. Over a median follow-up period of 15 months (range 9-29 months), 1 patient developed a stomal ulcer which improved with medical treatment. There were no patients with a body weight loss of ≥10 % relative to the preoperative body weight. No recurrence were experienced during the follow up period. Patients were free from postoperative diabetes mellitus. PSTD is a feasible and acceptable procedure in FAP patients with SC stage IV DP, in terms of surgical, pathological and clinical outcome. However, accumulation of the patients and long-term follow up study is necessary.

Japanese Society for Cancer of the Colon and Rectum (JSCCR) guidelines 2020 for the Clinical Practice of Hereditary Colorectal Cancer

“…In Japan, there is one single-center retrospective study regarding PSD (PSTD) performed in 10 patients with duodenal adenomas diagnosed with stage IV Spigelman classification. In terms of postoperative complications, four patients had pancreatic fistulas that have been managed through conservative treatment, and one patient had surgical site infection and cholangitis with low severity grade, suggesting that PSD (PSTD) is a feasible procedure [ 140 ].…”

“…In a summary of the studies on PSD between 1995 and 2012, 20% of all 96 patients underwent total duodenectomy, and 80% preserved their pyloric rings [ 140 ]. Whether or not total duodenectomy is necessary remains controversial, since there is no definite conclusion regarding the risk of carcinogenesis from the remnant duodenal mucosa after PSD with preservation of the pyloric ring.…”

“…Despite the preoperative diagnosis of duodenal adenomas, postoperative histological examinations reveal duodenal carcinoma in 9.6–30% of cases [ 140 , 142 ]. Therefore, it is crucial to perform preoperative assessments through careful observations of the duodenum and to select the appropriate surgical procedure for duodenal adenoma management among FAP patients with advanced Spigelman stages.…”

Endoscopic management of duodenal adenomatosis in familial adenomatous polyposis—A case‐based review

“…20 Other studies have reported even higher short-term morbidity rates (60%-76%). 50,51 Long-term morbidity (>30 days post-surgery) is also frequent, with pancreatitis (16%-21%) and exocrine pancreatic insufficiency (30%-60%) being the most common after PPD and PD, respectively. 49,52,53 The 30-day mortality rates vary from 2% to 29%.…”

Familial adenomatous polyposis: complex patient management

“…Endoskopické výkony by měly být prováděny preferenčně v celkové anestezii, endosko pisty zkušenými v technice a s maximálním důrazem na prevenci komplikací (léčba inhibitory protonové pumpy a zajištění pankreatického vývodu po papilek tomii stentem). Chirurgickými alternativami jsou polyp ektomie z duodenotomie a cefalická duodenopan kreatektomie, vysoce specializovaná centra provádějí i pankreas šetřící totální duodenektomii [22].…”