What is the MOST appropriate therapy for a small, well-circumscribed epithelioid sarcoma?

In the case of a small, well-circumscribed epithelioid sarcoma, the most appropriate therapy is typically surgery alone. This approach is favored because when such tumors are well-circumscribed, they often have clear margins that can be more easily resected. Complete surgical excision is usually curative for localized lesions, especially when there is no evidence of metastatic disease or significant invasion into surrounding tissues.

Choosing surgery as the primary treatment allows for direct removal of the tumor, minimizing the potential for recurrence. Other options, such as radiation therapy or systemic chemotherapy, might be reserved for cases where the tumor is more aggressive, larger, or has spread beyond the original site, but those are not typically first-line treatments for small, localized epithelioid sarcoma.

In contrast, while other therapies—like oral tazemetostat or combinations of surgery, radiation, and chemotherapy—can be useful in specific clinical situations or for tumors that are more advanced, they are not indicated as initial treatments for small, well-circumscribed tumors. Thus, the focus on surgery alone is crucial for achieving the best outcomes in such cases.