Diagnosis and treatment of extraabdominal desmoid fibromatosis

GMS Interdisciplinary Plastic and Reconstructive Surgery DGPW

Freiherr von Salis-Soglio, Georg¹  Heyde, Christoph-E.¹  Heinisch, Antje¹  Ghanem, Mohamed¹ 

[1] Department of Orthopaedic Surgery, University Hospital of Leipzig, Germany;

关键词: desmoid fibromatosis;    soft tissue tumors;    surgery;    local recurrence;   

DOI  :  10.3205/iprs000042

来源: DOAJ

【 摘 要 】

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. Ina 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.

【 授权许可】

Unknown