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

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

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

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

Summary for Physicians: Frontline Treatment Strategies for Advanced or Metastatic ES

Role of Conventional Chemotherapy: Conventional chemotherapy remains a cornerstone in the treatment of advanced or metastatic ES, particularly in patients with aggressive disease or those who are not candidates for surgery or radiation. Common chemotherapy options include ifosfamide and gemcitabine, often used in combination to improve efficacy. Chemotherapy is preferred in patients with rapidly progressing disease or those who require systemic treatment due to widespread metastasis. Patient factors such as performance status, prior treatment history, and potential for adverse effects guide the decision to select chemotherapy. The choice of regimen depends on the balance between efficacy and toxicity, with clinical considerations such as comorbidities influencing treatment decisions.

Role of Targeted Therapy: Targeted therapies are increasingly explored in the frontline setting, particularly for patients whose tumors exhibit specific molecular characteristics. Options may include agents targeting key pathways involved in ES, such as those related to the INI1 (SMARCB1) loss. Targeted therapy may be preferred in patients with molecular alterations that predict sensitivity to specific drugs. The decision to opt for targeted therapy is often based on the availability of biomarkers, the patient’s molecular profile, and previous treatment responses.

Biomarker testing, including genomic profiling, plays an integral role in selecting patients for targeted therapies, helping to identify those with actionable mutations or pathways amenable to treatment. These tests can provide insights into which therapies may be most effective and help personalize treatment decisions for individual patients.