The Desmoid Tumor Research Foundation

March 2018

Four different treatment strategies in aggressive fibromatosis: A systematic review

Author Information

Seinen JM(1), Niebling MG(1), Bastiaannet E(2), Pras B(3), Hoekstra HJ(1).

(1)Dept of Surgical Oncology, University of Groningen, University Medical Centre Groningen, The Netherlands. (2)Dept of Surgery and Department of Gerontology & Geriatrics, Leiden University Medical Centre, The Netherlands. (3)Dept of Radiation Oncology, University of Groningen, University Medical Centre Groningen, The Netherlands.

Publication

Clin Transl Radiat Oncol

Summary

The treatment approach for aggressive fibromatosis is changing. We compared the local control rate for surgery, surgery with radiotherapy, radiotherapy alone and a wait and see policy in a systematic review. A comprehensive search of the databases PubMed/Medline, Embase and Cochrane, of the medical literature published in 1999 till March 2017 was performed by two reviewers, including articles about extra abdominal aggressive fibromatosis without the genetical variants. A total of 671 studies were assessed for eligibility, and 37 studies were included for analysis, representing 2780 patients. The local control rates for surgery alone, surgery and radiotherapy, radiotherapy alone and observation were 75%, 78%, 85% and 78%, respectively. For patients with recurrent disease observation had a better local control rate than surgery alone (p=0.001). In the observation group, stabilization of the tumor was seen in median 14 (range 12-35) months. The time to local recurrence in the treatment group was median 17 (range, 11-52) months. A watchful conservative first line approach with just observation and closely monitoring, by means of physical examination and MRI, appears to be justified in a subgroup of patients without clinical symptoms and no possible health hazards if the tumor would progress.

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