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United European Gastroenterol J. 2017 Aug;5(5):735-741. doi: 10.1177/2050640616678150. Epub 2016 Oct 28.

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

United European gastroenterology journal

T Walter, C Zhenzhen Wang, O Guillaud, E Cotte, A Pasquer, O Vinet, G Poncet, T Ponchon, J-C Saurin

Affiliations

  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.

PMID: 28815038 PMCID: PMC5548349 DOI: 10.1177/2050640616678150

Abstract

BACKGROUND: Desmoid tumours represent a major complication of familial adenomatous polyposis. 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.

METHODS AND PATIENTS: 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. Response to treatment of desmoid tumours was assessed by Response Evaluation Criteria In Solid Tumor (RECIST) criteria.

RESULTS: A total of 180 patients with familial adenomatous polyposis were included with a median follow-up of 19 years since diagnosis. Thirty-one (17%) patients developed 58 desmoid tumours, a median (range) 4.7 (0.8-41.6) years after their diagnosis of familial adenomatous 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,

CONCLUSION: 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.

Keywords: Desmoid tumour; familial adenomatous polyposis; risk factor; surgery; treatment

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