Diagnosis and Treatment of Extraabdominal Desmoid Fibromatosis

Overview

Journal GMS Interdiscip Plast Reconstr Surg DGPW

Specialty General Surgery

Date 2015 Oct 28

PMID 26504712

Citations 1

Authors

Mohamed Ghanem

Antje Heinisch

Christoph-E Heyde

Georg Freiherr von Salis-Soglio

Affiliations

Soon will be listed here.

Abstract

Introduction: The desmoid fibromatosis is a very rare connective tissue disease which is recognized as semimalignant. The aim of this work is to review the relevant literature and to analyze the management of our patient collective.

Material And Method: Surgery was performed on 7 patients with extraabdominal desmoid fibromatosis between August 1998 and May 2007. MRI examination as well as biopsy was carried out in all cases. All patients were operated on; the mean follow up was 4 years (1-7). Upon follow up, every patient has undergone clinical and MRI examination.

Results: The results show that we have achieved R0 resection in 4 cases and R1 in two cases and Rx in one case. In 4 patients, no recurrence was observed after the single surgery performed in our hospital. In 2 patients a single revision surgery was performed in each case and yielded no further recurrence. In only one case, multiple surgeries (one primary and two revision surgeries) were necessary, after which no recurrence was reported.

Conclusion: The early diagnosis of the disease is of utmost importance to the success of the outcome. MRI examination and biopsy are mandatory. Surgery is the therapy of choice. The recurrence rate is high and is linked to the difficulty of recognition of the exact infiltrative extent of the tumour. This necessitates a close follow-up.

Citing Articles

Extra-Abdominal Desmoid-Type Fibromatosis Mimicking Myxofibrosarcoma.

Kim Y, Yoon Y, Park H J Belg Soc Radiol. 2022; 106(1):81.

PMID: 36213374 PMC: 9504096. DOI: 10.5334/jbsr.2848.

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