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Sarcoma
Volume 2013 (2013), Article ID 256280, 6 pages
http://dx.doi.org/10.1155/2013/256280
Clinical Study

Low-Grade Fibromyxoid Sarcoma: Incidence, Treatment Strategy of Metastases, and Clinical Significance of the FUS Gene

1Sarcoma Centre of Aarhus University Hospital, Denmark
2Department of Experimental Clinical Oncology, Aarhus University Hospital, Noerrebrogade 44, 8000 Aarhus C, Denmark
3Department of Pathology, Aarhus University Hospital, Noerrebrogade 44, 8000 Aarhus C, Denmark
4Department of Orthopaedic Surgery E5, Aarhus University Hospital, Noerrebrogade 44, 8000 Aarhus C, Denmark
5Department of Oncology, Aarhus University Hospital, Noerrebrogade 44, 8000 Aarhus C, Denmark

Received 25 February 2013; Accepted 13 May 2013

Academic Editor: R. Pollock

Copyright © 2013 Katja Maretty-Nielsen et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Aim. The aim of this study was to assess the incidence of low-grade fibromyxoid sarcoma (LGFMS), present treatment results of metastatic LGFMS, and investigate the clinical significance of the FUS gene rearrangement. Methods. This study included 14 consecutive LGFMS patients treated at the Aarhus Sarcoma Centre in 1979–2010. Fluorescent in situ hybridization (FISH) analysis for FUS break-apart was performed for all patients. Results. The incidence of LGFMS was 0.18 per million, representing 0.6% of all soft tissue sarcomas. Four patients needed multiple biopsies/resections before the correct diagnosis was made. Four patients experienced local recurrence, and three patients developed metastases. The treatment of metastatic LGFMS varied from multiagent chemotherapy to repeated, selective surgery of operable metastases. The best response to chemotherapy was short-term stabilization of disease progression, seen with Trabectedin. The prevalence of the FUS break-apart was 21.4%. We found no significant difference in clinical characteristics and outcomes in correlation with the FUS break-apart. Conclusion. LGFMS is a rare disease with multiple challenges. The FUS break-apart was not associated with local recurrence or metastases in our study. To date the only treatment resulting in disease-free periods is surgery; however further investigation into the management of metastatic LGFMS is necessary.