Low-grade Fibromyxoid Sarcoma: Treatment Outcomes and Efficacy of Chemotherapy

Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma subtype with a generally indolent pattern of clinical behaviour, but treatments for advanced disease are limited. A retrospective search of a prospectively maintained institutional database identified 102 patients treated from December 1994 to...

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Published inIn vivo (Athens) Vol. 34; no. 1; pp. 239 - 245
Main Authors Chamberlain, Florence, Engelmann, Bodil, Al-Muderis, Omar, Messiou, Christina, Thway, Khin, Miah, Aisha, Zaidi, Shane, Constantinidou, Anastasia, Benson, Charlotte, Gennatas, Spyridon, Jones, Robin L
Format Journal Article
LanguageEnglish
Published Greece International Institute of Anticancer Research 01.01.2020
Subjects
Low-grade fibromyxoid sarcoma
radiotherapy
LGFMS
chemotherapy
chromosomal translocation
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Summary:Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma subtype with a generally indolent pattern of clinical behaviour, but treatments for advanced disease are limited. A retrospective search of a prospectively maintained institutional database identified 102 patients treated from December 1994 to August 2018. We evaluated the outcome of patients and the efficacy and safety of non-surgical therapies in LGFMS. Ninety-four out of 102 (92.2%) underwent primary resection, seven (6.9%) were treated with systemic therapy and one (1.0%) is currently being treated with pre-operative radiotherapy. The RECIST 1.1 response rate to first-line chemotherapy was 0%, and median progression-free survival was 1.84 months (95% confidence intervaI=0.10-3.6 months). Conventional systemic therapy has limited efficacy in advanced LGFMS.
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ISSN:0258-851X
1791-7549
DOI:10.21873/invivo.11766