Tazemetostat combined PD-1 inhibitor achieving prolonged survival in primary proximal epithelioid sarcoma: a case report and review of the literature.
- 1Department of Medical Oncology, Zhongshan Hospital, Fudan University, No. 180, Fenglin Road, Xuhui District, Shanghai, China.
- 2Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China.
- 3Department of Medical Oncology, Zhongshan Hospital, Fudan University, No. 180, Fenglin Road, Xuhui District, Shanghai, China. [email protected].
Abstract
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.
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BibTeX
@article{li2026tazemetostat,
title = {{Tazemetostat combined PD-1 inhibitor achieving prolonged survival in primary proximal epithelioid sarcoma: a case report and review of the literature.}},
author = {Wei Li and Chenlu Zhang and Yang You and Xi Guo and Rongkui Luo and Yuhong Zhou},
journal = {Journal of medical case reports},
year = {2026},
month = aug,
doi = {10.1186/s13256-026-06312-4},
url = {https://europepmc.org/article/MED/42958422},
note = {CC BY},
}
RIS
TY - JOUR
TI - Tazemetostat combined PD-1 inhibitor achieving prolonged survival in primary proximal epithelioid sarcoma: a case report and review of the literature.
AU - Wei Li
AU - Chenlu Zhang
AU - Yang You
AU - Xi Guo
AU - Rongkui Luo
AU - Yuhong Zhou
PY - 2026
DA - 2026/08/03
JO - Journal of medical case reports
DO - 10.1186/s13256-026-06312-4
UR - https://europepmc.org/article/MED/42958422
AB - 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.
SN - 1752-1947
ER -