Cost-Effectiveness of Prophylactic Surgery for Duodenal Cancer in Familial Adenomatous Polyposis

Abstract

Background: Duodenal cancer is the leading cause of cancer death in familial adenomatous polyposis after colorectal cancer. The lifetime risk for developing duodenal cancer is 4% to 10%. Current treatment guidelines recommend endoscopic surveillance with a prophylactic pancreaticoduodenectomy in advanced duodenal polyposis, defined using the Spigelman staging system. Because no clinical trials have assessed this recommendation, a modeling approach was used to evaluate the cost-effectiveness of various treatment strategies. Methods: A Markov model was constructed to estimate the life expectancy and cost of three different strategies: pancreaticoduodenectomy at Spigelman stage III, pancreaticoduodenectomy at Spigelman stage IV, and pancreaticoduodenectomy at cancer diagnosis. A cohort of 30-year-old familial adenomatous polyposis patients with total colectomies was simulated until age 80. The

Updated European guidelines for clinical management of familial adenomatous polyposis (FAP), MUTYH-associated polyposis (MAP), gastric adenocarcinoma, proximal polyposis of the stomach (GAPPS) and other rare adenomatous polyposis syndromes: a joint EHTG-ESCP revision

“…Interestingly, patients who are downstaged from stage IV to stages I, II or III have a significantly higher risk of duodenal cancer compared to patients who naturally progress to stages I, II or III 112 , 115 . However, the current Spigelman stage does not take this information into account 102 , 115 , 119 , 148 . Other concerns regarding the Spigelman stage include its application to the neo-duodenum after duodenal surgery 109 , its validity when chromoendoscopy significantly increases the duodenal polyp count and, consequently, the Spigelman stage 122 , 149 , and whether additional duodenal cancer risk factors should be included 104 , 116 , 121 , 127 .…”

“…Duodenal surgery is associated with significant short-term mortality (about 5%) and morbidity (30–63%) 142 , 152–155 , so it should be offered to carefully selected patients 141 , 156 . Nevertheless, there should be a low threshold to escalate to duodenal surgery once the disease becomes no longer manageable endoscopically 139 , 141 , 142 , 146 , 148 , 150 , 157 , 158 . Several studies have reported a high risk (range 8–37%) of unsuspected duodenal cancers that are diagnosed only after histological review 108 , 109 , 139 , 141 , 142 , 146–148 , 150 , 158 .…”

“…It then become popular for several reasons, including the ease of computation, the replicability and the ability to monitor disease progression over time and after endoscopy therapy 99 , 102–104 , 111 , 119 , 121 , 122 . Therefore, there is a sufficiently large body of evidence to recommend its use in clinical practice 104 , 118 , 122 , 148 . However, it has some weaknesses that warrant careful scrutiny, including a suboptimal sensitivity and specificity for predicting the risk of duodenal and papillary cancer.…”

“…This suggests a more aggressive course for patients who develop stage IV polyposis during their lifetime 112 , 115 . However, the Spigelman stage treats all patients with stages I, II or III equally, regardless of downstaging 102 , 115 , 119 , 148 . Moreover, it is unclear how the Spigelman stage should be applied to the neo-duodenum after duodenal surgery 109 .…”

“…Therefore, endoscopic downstaging of duodenal polyposis is the primary treatment strategy. When endoscopic treatment becomes no longer feasible, duodenal surgery may be considered for Spigelman stages III/IV 148 , 150 , 157 . Duodenal surgery has been traditionally offered to patients with stage IV duodenal polyposis, but this approach must be carefully considered 98 , 139 , 141 , 142 , 146–148 , 150 , 152 .…”