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Background <p>Epithelioid sarcoma (ES) is a rare mesenchymal tumor categorized into proximal (axial) and classical (distal) types. In general, proximal ES come alone with limited treatment options and poor prognosis. Herein, we present the case of a proximal ES treated with a tazemetostat-containing regimen and achieved a favorable response in the third-line setting.</p> Case description <p>A 44-year-old Han woman was noticed a left pelvic floor nodule by computed tomography (CT) scan, and histologically test result confirmed her diagnosis as epithelioid sarcoma (proximal type). Cell staining indicates a negative expression of INI1 but positive expression of PD-L1. Ifosfamide and adriamycin (IA) were introduced as first-line treatment because of multidisciplinary treatment (MDT) of soft-tissue tumor considered tumor invasion is extensive, not eligible to complete surgical resection. Unfortunately, IA was discontinued due to patient intolerance (severe myelosuppression). Anlotinib was used as second-line treatment after rapid disease progression and also discontinued due to vaginal hemorrhage. After the pathology diagnosis, the combination therapy of tislelizumab and tazemetostat was introduced. A significant tumor shrinkage was noticed after 21&#xa0;months of follow-up, which achieved and continue to maintain a partial response (PR) with further tumor reduction.</p> Conclusions <p>This case first reported a favorable response of primary proximal epithelioid sarcoma treated with EZH2 inhibitor tazemetostat and PD-1 inhibitor tislelizumab, suggesting a potential and well-tolerated treatment regimen for this rare disease.</p>

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Tazemetostat combined PD-1 inhibitor achieving prolonged survival in primary proximal epithelioid sarcoma: a case report and review of the literature

  • 期刊论文

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  • 发表日期：

    2026年8月3日

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  • Wei Li,
  • Chenlu Zhang,
  • Yang You,
  • Xi Guo,
  • Rongkui Luo,
  • Yuhong Zhou

摘要

Background

Epithelioid sarcoma (ES) is a rare mesenchymal tumor categorized into proximal (axial) and classical (distal) types. In general, proximal ES come alone with limited treatment options and poor prognosis. Herein, we present the case of a proximal ES treated with a tazemetostat-containing regimen and achieved a favorable response in the third-line setting.

Case description

A 44-year-old Han woman was noticed a left pelvic floor nodule by computed tomography (CT) scan, and histologically test result confirmed her diagnosis as epithelioid sarcoma (proximal type). Cell staining indicates a negative expression of INI1 but positive expression of PD-L1. Ifosfamide and adriamycin (IA) were introduced as first-line treatment because of multidisciplinary treatment (MDT) of soft-tissue tumor considered tumor invasion is extensive, not eligible to complete surgical resection. Unfortunately, IA was discontinued due to patient intolerance (severe myelosuppression). Anlotinib was used as second-line treatment after rapid disease progression and also discontinued due to vaginal hemorrhage. After the pathology diagnosis, the combination therapy of tislelizumab and tazemetostat was introduced. A significant tumor shrinkage was noticed after 21 months of follow-up, which achieved and continue to maintain a partial response (PR) with further tumor reduction.

Conclusions

This case first reported a favorable response of primary proximal epithelioid sarcoma treated with EZH2 inhibitor tazemetostat and PD-1 inhibitor tislelizumab, suggesting a potential and well-tolerated treatment regimen for this rare disease.