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Optimizing the Care Pathway for Advanced Epithelioid Sarcoma: Diagnosis, Treatment, and Beyond : Episode 7

Navigating Frontline Therapy in Epithelioid Sarcoma: Key Clinical Considerations and Shared Decision-Making

May 9, 2025
By Neeta Somaiah, MD
Edwin Choy, MD, PhD
  • Brian Rubin, MD, PhD

Opinion
Video

Panelists discuss how a multimodal frontline treatment approach for epithelioid sarcoma (ES)—combining surgery, radiation, and systemic therapies—should be tailored to tumor resectability, disease stage, and patient-specific factors such as molecular characteristics and overall health.

EP: 1.Epithelioid Sarcoma: Overview and Presentation

EP: 2.Epithelioid Sarcoma: Histological Features and Differential Diagnoses

EP: 3.Confirming Epithelioid Sarcoma: Diagnostic Approach, Imaging, and Molecular Markers

EP: 4.Navigating Diagnostic Challenges in Epithelioid Sarcoma: Multidisciplinary Strategies to Optimize Diagnosis

EP: 5.A 62-Year-Old Woman With Epithelioid Sarcoma Treated With Tazemetostat

EP: 6.Management Strategies for Localized Epithelioid Sarcoma: The Role of Neoadjuvant and Adjuvant Therapies

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EP: 7.Navigating Frontline Therapy in Epithelioid Sarcoma: Key Clinical Considerations and Shared Decision-Making

EP: 8.Managing Epithelioid Sarcoma: Follow-Up Strategies and Treatment Decisions for Stable Disease vs Progression

Summary for Physicians: Frontline Treatment Approach for ES

The frontline treatment of ES typically involves a multimodal approach, incorporating surgery, radiation, and systemic therapy. The choice of therapy depends on tumor location, resectability, and disease stage.

Role of Surgery and Radiation Therapy: Surgery is the mainstay for localized ES, with complete surgical resection being the preferred option for tumors that are amenable to this approach. Radiation therapy may be used as an adjunct to surgery, especially in cases where clear margins cannot be achieved, or in patients with recurrent or inoperable disease. Radiation can help reduce the risk of local recurrence and improve outcomes when surgery alone is insufficient.

Role of Systemic Therapy: Systemic therapy is preferred in cases of advanced or metastatic ES, where the disease is not amenable to surgery or radiation. Chemotherapy, often with agents like ifosfamide or gemcitabine, is commonly used as a frontline treatment for metastatic disease or in patients with high-risk features. Systemic therapy may also be indicated in cases of large, locally advanced tumors that cannot be fully resected. Decisions regarding systemic therapy are guided by factors such as the extent of disease, patient performance status, molecular characteristics of the tumor, and potential treatment-related toxicities.

In summary, systemic therapies are typically reserved for advanced stages or when surgery and radiation are not feasible, and therapy selection is influenced by the individual patient’s disease characteristics and overall health.

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