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BMC Musculoskelet Disord. 2018 Jan 5;19(1):2. doi: 10.1186/s12891-017-1924-3.

Desmoid Tumours of the extremity and trunk. A retrospective study of 44 patients.

Author information

1
Musculoskeletal Oncology, Department of Orthopaedic Surgery, Physical Medicine and Rehabilitation, Campus Grosshadern, University Hospital, LMU Munich, Marchioninistr. 15, 81377, Munich, Germany.
2
Institute of Radiology, Campus Grosshadern, University Hospital, LMU Munich, Marchioninistr. 15, 81377, Munich, Germany.
3
Institute of Institute of Pathology, Campus Grosshadern, University Hospital, LMU Munich, Marchioninistr. 15, 81377, Munich, Germany.
4
Department of Medicine III, Campus Grosshadern, University Hospital, LMU Munich, Marchioninistr. 15, 81377, Munich, Germany.
5
Department of Radiation Oncology, Campus Grosshadern, University Hospital, LMU Munich, Marchioninistr. 15, 81377, Munich, Germany.
6
CCU Radiation Oncology, German Cancer Research Center (DKFZ), Heidelberg, All authors are part of the SarKUM, the Bone and Soft Tissue Tumor Center of the University Hospital, Ludwig-Maximilians-University Munich, Munich, Germany.
7
Musculoskeletal Oncology, Department of Orthopaedic Surgery, Physical Medicine and Rehabilitation, Campus Grosshadern, University Hospital, LMU Munich, Marchioninistr. 15, 81377, Munich, Germany. hans_roland.duerr@med.uni-muenchen.de.

Abstract

BACKGROUND:

Desmoid-type fibromatosis (DF) is a aggressive (myo)fibroblastic neoplasm with an infiltrative growth and a tendency to local recurrence. Resection of the tumour and/or radiation were proposed as principal treatment. The aim of this retrospective study was to analyze the local control rates focusing on the effect of surgical margins and radiotherapy.

METHODS:

From 1981 to 2014, 44 patients had been treated. Fifty four therapies had been applied, in 50 cases surgery +/- radiation therapy, NSAIDs or chemotherapy. In 4 cases a conservative approach was chosen. Thirty seven patients had primary, 17 recurrent disease. Endpoint was either local recurrence (LR), progression of residual disease or rare non-metastatic secondary lesions at the same extremity.

RESULTS:

The mean age was 39,4 years. In 17 cases a R0, in 27 a R1 and in 6 cases a R2 resection was achieved. Four patients were treated conservatively. All together in 21 cases radiotherapy, in 5 NSAIDs, in 3 imatinib and in 2 cases each tamoxifen or chemotherapy had been applied. The median follow-up was 119 months. 5-year recurrence free survival after resection was 78%. 10 (20.4%) patients developed LR between 5 and 42 months after therapy. Recurrent disease was a negative factor on LR. Margins, radiotherapy, sex, or size of the tumour had no significant impact on LR. Patients younger than 40 years had a significant higher risk of LR.

CONCLUSIONS:

Surgical margins are less important than keeping function. Radiotherapy might be an option in unresectable lesions, the role of adjuvant radiotherapy is controversially discussed.

KEYWORDS:

Desmoid; Extra-abdominal; Fibromatosis; Prognosis; Recurrence

PMID:
29304783
PMCID:
PMC5756424
DOI:
10.1186/s12891-017-1924-3
[Indexed for MEDLINE]
Free PMC Article

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