Systemic Treatment for Adults with Synovial Sarcoma.

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Authors

Desar, IME
Fleuren, EDG
van der Graaf, WTA

Document Type

Journal Article

Date

2018-03-07

Date Accepted

Abstract

Opinion statement 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.

Citation

Current treatment options in oncology, 2018, 19 (2), pp. 13 - ?

Source Title

Publisher

ISSN

1527-2729

eISSN

1534-6277

Collections

Research Team

Clinical and Translational Sarcoma

Notes