The information provided in this activity is for accredited continuing education purposes only and is not meant to substitute for the independent clinical judgment of a health care professional relative to diagnostic, treatment, or management options for a specific patient's medical condition. Wide surgical excision of the tumor with neoadjuvant or adjuvant radiation treatment of the tumor bed is the primary treatment of choice in most cases of ES.2,7,8 Depending on the location and extent of local tumor invasion, limb-sparing surgery may or may not be feasible, with amputation being the alternative.7,8 Perioperative radiation treatment effectively reduces rates of local recurrence, and it is recommended in all but the lowest stages of ES.7,8 Disease that recurs, metastasizes, or is diagnosed at an advanced stage is often treated with some form of systemic therapy (eg, traditional chemotherapy or targeted medication).7 In addition to disease factors, individual patient characteristics must be considered, and shared decision-making is encouraged. In 2020, the medication received accelerated approval and orphan drug status from the FDA for treating advanced ES based on results of a phase 2 clinical trial.12 The study enrolled 62 patients; investigators found an objective response rate of 15%, with 26% of patients having disease control at 32 weeks of follow-up.12 This has led to NCCN guidelines listing tazemetostat as the preferred treatment for recurrent, metastatic, or locally advanced and unresectable ES.7 Additionally, tazemetostat is still being investigated in combination with doxorubicin as a first-line systemic therapy for advanced ES.13 Key References 1. According to current data and guideline recommendations, what is the next best step in the management of this patient's ES at this time? A. Doxorubicin B. Gemcitabine plus docetaxel C. Larotrectinib, 100 mg, orally twice daily D. Tazemetostat, 800 mg, orally twice daily E. Tazemetostat, 800 mg, orally twice daily plus doxorubicin To learn more about this topic, including information on the identification, diagnosis, and management of epithelioid sarcoma, go to https://www.gotoper.com/cac25es-activity CME Provider Contact information Physicians' Education Resource®, LLC 2 Commerce Drive, Suite 110, Cranbury, NJ 08512 Toll-Free: 888-949-0045 Local: 609-378-3701 Fax: 609-257-0705 info@gotoper.com The experts data:image/svg+xml,%3csvg%20xmlns=%27http://