Expert Perspectives on the Assessment and Management of Advanced or Metastatic Epithelioid Sarcoma in the Second-Line Setting and Beyond

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Panelists discuss how assessing disease progression, utilizing molecular testing (eg, INI1 loss), and considering prior treatment responses are crucial for selecting appropriate second-line therapies, with targeted therapies and immunotherapies offering promising options for managing advanced or metastatic epithelioid sarcoma (ES).

Summary for Physicians: Assessing and Managing Advanced or Metastatic ES for Subsequent Therapy

When assessing patients with advanced or metastatic ES for second-line (2L) therapy, the evaluation of disease progression is guided by clinical assessment, imaging studies (eg, CT, MRI), and symptom monitoring. Disease progression is typically defined by radiographic evidence of new or enlarging lesions, clinical deterioration, or worsening symptoms. The decision to move to 2L therapy is influenced by disease characteristics, prior treatment response, and the patient’s overall condition.

Molecular testing plays an increasingly important role in guiding therapy selection. The loss of INI1 (SMARCB1) is a key molecular marker for diagnosis and can help guide treatment decisions. In practice, genomic profiling may reveal actionable mutations or sensitivities to specific targeted therapies or immunotherapies, which can be integrated into second-line treatment strategies.

For patients with disease progression after frontline chemotherapy or targeted therapy, treatment options include chemotherapy agents such as ifosfamide or gemcitabine, and in some cases, newer targeted therapies or immunotherapies may be considered. Treatment selection is based on factors such as prior treatment efficacy, patient performance status, and the molecular features of the tumor, which could indicate susceptibility to specific therapies. Decisions are also guided by the potential for adverse effects and the patient’s quality of life.

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