OR13: Surveillance of Duodenal Polyposis in Familial Adenomatous Polyposis: Should the Spigelman score be modified?

Isabelle Sourrouille1,Jérémie H Lefèvre1,Conor Shields2, Chrystelle Colas3, Jerôme Bellanger4, Benoît Desaint5, Emmanuel Tiret1, Yann Parc1.

1. Department of Digestive Surgery, Hôpital Saint-Antoine. 2. Mater Misericordiae University Hospital, Dublin, Ireland. 3. Laboratory of Angiogenetics and Oncogenetics, Hôpital Pitié-Salpétrière. 4. Centre de prise en charge multidisciplinaire des personnes predisposes héréditairement au cancer colorectal, Hôpital Saint-Antoine. 5. Department of Digestive Endoscopy, Hôpital Saint-Antoine

Aim

Duodenal polyposis (DP) is a manifestation of Adenomatous Polyposis (AP) which predisposes to duodenal or ampullary adenocarcinoma (ADC). DP is monitored by upper GI endoscopies (UGIE) and may require iterative resections and prophylactic radical surgical treatment (RST), when malignancy is threatening. Evaluate severity scoring for surveillance and treatment in a large series of DP.

Method

From 1982 to 2014, every patient surveyed by UGIE for DP was included.

Results

We performed 1912 UGIE in 437 patients (median 3, IQR [2;6]). Genes involved were APC (n=274, 62.7%) and MYH (n=21, 4.8%). First UGIE (median age 32[21;44]) revealed DP in 190 (43.5%). Rates of low grade dysplasia (LGD), high grade dysplasia (HGD), and ADC at 5 years were 65%[61.7-66.9], 12.1%[10.3-13.9] and 2.4%[1.5-3.3]. while 10 year rates were 75.8%[73.1-78.5], 20.8%[18.2-23.4] and 5.4%[3.8-7.0] respectively. The rate of ampullary abnormalities rose during surveillance, from 18.3% at the first UGIE to 47.4% at the fourth. Predictive factors for HGD were age at first UGIE, type and age of colorectal surgery, Spigelman score, presence of an ampullary abnormality, and the number of endoscopic treatments. In multivariate analysis, only age at first UGIE and presence of an ampullary abnormality were independent predictive factors. Conservative treatment was performed in 103 patients (159 endoscopic resections, 17 surgical), while RST (Whipple procedure or duodenectomy) was required in 52 (median age 47.5[43-57.3]) because of HGD or unresectable lesions. Histological analysis after RST showed HGD in 30 patients and ADC in 11 (4 patients had lymph node involvement).

Conclusion

Over 20% of patients develop HGD with DP after 10 years. Iterative endoscopic resections allow extended control but surgery remains necessary in 12% of the patients, and happens too late in many cases; 20% had developed ADC, while 8% exhibited malignancy with lymph node involvement. The trigger for prophylactic surgery requires a more accurate predictive score. Modifying the Spigelman score by accounting for ampullary abnormalities should be considered.

Top