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Annals of Surgical Oncology
Published in: Annals of Surgical Oncology 6/2018

01-06-2018 | Bone and Soft Tissue Sarcomas

Systematic Review of Clinical Outcomes Following Various Treatment Options for Patients with Extraabdominal Desmoid Tumors

Authors: Kortnye Smith, MBBS, FRACP, Jayesh Desai, MBBS, FRACP, Smaro Lazarakis, B.Ed.(Sec)-Lib, David Gyorki, MBBS, MD, FRACS

Published in: Annals of Surgical Oncology | Issue 6/2018

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Abstract

Background

Desmoid tumors (DT) are rare clonal proliferations that arise from mesenchymal cells. These tumors do not metastasize but are locally aggressive, and their growth may lead to significant morbidity. Their clinical course is both variable and unpredictable; tumors may rapidly progress but in other instances remain stable or regress without intervention.

Aims

To examine current treatment of DT and assist with decision-making at time of presentation.

Methods

A literature search was conducted of MEDLINE and Cochrane databases for published studies (1995–July 2015) using the search terms fibromatosis aggressive, desmoid with drug therapy, radiation therapy, prevention and control, radiotherapy, surgery, and therapy. Articles were categorized as surgery, radiation, surgery + radiation, systemic therapy, and front-line observation. Articles were included if they reported a retrospective or prospective comparative or observational study with an analyzed sample size of 10 patients or more with confirmed diagnosis of desmoid tumor and described one of the following clinical outcomes: relapse- or progression-free survival, local control rate, response rate.

Results

258 articles were reviewed; following screening for eligibility, 54 were identified; following full-text screen, 31 were included in final evaluation. The control rate for patients treated with a “wait and see” observational approach compared favorably with management with surgery and resulted in disease control rates of between 60 and 92%.

Conclusions

Decision-making in this rare tumor is complicated by the range of treatment options available. Our evidence supports use of an upfront observational approach.
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Metadata
Title
Systematic Review of Clinical Outcomes Following Various Treatment Options for Patients with Extraabdominal Desmoid Tumors
Authors
Kortnye Smith, MBBS, FRACP
Jayesh Desai, MBBS, FRACP
Smaro Lazarakis, B.Ed.(Sec)-Lib
David Gyorki, MBBS, MD, FRACS
Publication date
01-06-2018
Publisher
Springer International Publishing
Published in
Annals of Surgical Oncology / Issue 6/2018
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-018-6408-7

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