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Review
. 2018 Mar 7;19(2):13.
doi: 10.1007/s11864-018-0525-1.

Systemic Treatment for Adults with Synovial Sarcoma

Ingrid M E Desar  1 , Emmy D G Fleuren  2 , Winette T A van der Graaf  3   4
Affiliations
Review

Systemic Treatment for Adults with Synovial Sarcoma

Ingrid M E Desar et al. Curr Treat Options Oncol. .

Abstract

Synovial sarcoma (SS) is a rare, yet highly malignant, type of soft tissue sarcoma (STS), for which survival has not improved significantly during the past years. In this review, we focus on systemic treatment in adults. Compared to other STS, SS are relatively chemosensitive. Ifosfamide and ifosfamide combinations are active in different lines of treatment. In high-risk extremity and chest wall STS, neoadjuvant doxorubicin and ifosfamide has shown as much activity as high-dose ifosfamide. There are indications that combination chemotherapy with doxorubicin and ifosfamide in this setting improves outcome. In the first-line metastatic setting, combination treatment with doxorubicin and ifosfamide is a preferred option in fit patients, while in other patients, sequential doxorubicin and ifosfamide can be considered. In second and later lines, pazopanib and trabectedin have shown activity. Many new approaches to treat metastatic SS are currently under investigation, both preclinical as well as clinical, including other receptor tyrosine kinase inhibitors, epigenetic modulators, compounds interfering with DNA damage response (DDR), and immunotherapy.

Keywords: Chemotherapy; Immunotherapy; Synovial sarcoma; Targeted therapy.

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Conflict of interest statement

Conflict of interest

Ingrid M.E. Desar has served on advisory boards for Lilly and Eisai. Emmy D.G. Fleuren declares that she has no conflict of interest. Winette T.A. van der Graaf has received research funding through a grant from Novartis.

Human and animal rights and informed consent

All reported studies/experiments with human or animal subjects performed by the authors have been previously published and complied with all applicable ethical standards (including the Helsinki declaration and its amendments, institutional/national research committee standards, and international/national/institutional guidelines).

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