The Desmoid Tumor Research Foundation

October 2017

Management of desmoid tumours: A large national database of familial adenomatous patients shows a link to colectomy modalities and low efficacy of medical treatments

Author Information

Walter T(1), Zhenzhen Wang C(1)(2), Guillaud O(1), Cotte E(3), Pasquer A(4), Vinet O(1), Poncet G(4), Ponchon T(1), Saurin JC(1).

(1)Gastroenterology Department, Edouard Herriot Hospital, Lyon, France.(2)Ruijin Hospital, Shanghai Jiaotong University, China.(3)Surgery Department, Lyon Sud Hospital, Lyon, France.(4)Surgery Department, Edouard Herriot Hospital, Lyon, France.

Publication

United Eur Gastroenterol J

Summary

Our aims were to study the factors associated with the development of desmoid tumours in familial adenomatous polyposis patients, and to describe presentation and management of desmoid tumours. We reviewed all patients with familial adenomatous polyposis followed at our institution between 1965-2013, with either identified adenomatous polyposis coli gene mutation, or a personal and family history suggesting adenomatous polyposis coli-related polyposis. The only factor significantly associated with occurrence of desmoid tumours was the type of surgery: 12 (12%) desmoid tumours in 104 patients treated by colectomy, versus 19 (25%) desmoid tumours in 76 patients treated by proctocolectomy, p_=_0.027. If confirmed, colectomy (versus proctocolectomy) should be performed in adenomatous polyposis coli-related familial adenomatous polyposis patients to avoid desmoid tumours. We show that there is a high prevalence of post-surgical recurrence and the low efficacy of available medical treatments for desmoid tumours.

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