Epithelioid sarcoma medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]

Overview

Doxorubicin is the drug of choice.
The gold standard for chemotherapy is a combination of doxorubicin and ifosfamide. However, recent studies have suggested that the addition of ifosfamide to doxorubicin does not necessarily lead to an increase in overall survival.^[1] Etoposide, vincristine, dactinomycin, and cyclophosphamide have also traditionally been given.^[2] Newer chemotherapies, such as gemcitabine and pazopanib, are currently being tested in clinical trials.

Radiation Therapy was used for limb salvage therapy.
Radiation therapy is also a treatment option when tumors are deemed inoperable or wide surgical margins are not achievable. Radiation therapy in combination with chemotherapy has so far resulted in only minimal improvements to response rates. Trials with brachytherapy (an internal radiation treatment that delivers a high dose of radiation directly to the tumor and is thought to have fewer long-term side effects) have produced some positive results.^{[citation needed]}

↑ Judson, Ian; Verweij, Jaap; Gelderblom, Hans; Hartmann, Jörg T; Schöffski, Patrick; Blay, Jean-Yves; Kerst, J Martijn; Sufliarsky, Josef; Whelan, Jeremy; Hohenberger, Peter; Krarup-Hansen, Anders; Alcindor, Thierry; Marreaud, Sandrine; Litière, Saskia; Hermans, Catherine; Fisher, Cyril; Hogendoorn, Pancras C W; dei Tos, A Paolo; van der Graaf, Winette T A (2014). "Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a randomised controlled phase 3 trial". The Lancet Oncology. 15 (4): 415–23. doi:10.1016/S1470-2045(14)70063-4. PMID 24618336.
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