{
  "abstract": "Background\n\nEpithelioid 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\n\nA 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\n\nThis 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.",
  "abstract_shown": true,
  "affiliations": [
    "Department of Medical Oncology, Zhongshan Hospital, Fudan University, No. 180, Fenglin Road, Xuhui District, Shanghai, China.",
    "Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China.",
    "Department of Medical Oncology, Zhongshan Hospital, Fudan University, No. 180, Fenglin Road, Xuhui District, Shanghai, China. zhou.yuhong@zs-hospital.sh.cn."
  ],
  "article_type": "review",
  "arxiv": null,
  "author_total": 6,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Wei Li"
    },
    {
      "affs": [
        0
      ],
      "name": "Chenlu Zhang"
    },
    {
      "affs": [
        0
      ],
      "name": "Yang You"
    },
    {
      "affs": [
        0
      ],
      "name": "Xi Guo"
    },
    {
      "affs": [
        1
      ],
      "name": "Rongkui Luo"
    },
    {
      "affs": [
        2
      ],
      "name": "Yuhong Zhou"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/2b0eqfkmzh3eq.bib",
  "cited_by": 0,
  "doi": "10.1186/s13256-026-06312-4",
  "fulltext_url": "https://europepmc.org/articles/PMC13721315",
  "id": "2b0eqfkmzh3eq",
  "issns": [
    "1752-1947"
  ],
  "landing_url": "https://europepmc.org/article/MED/42958422",
  "language": "eng",
  "licence": "CC-BY",
  "licence_label": "CC BY",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "heading",
      "Case Report"
    ],
    [
      "openalex",
      "2740|Sarcoma Diagnosis and Treatment"
    ]
  ],
  "openalex": "W7172219903",
  "pdf_url": "https://europepmc.org/articles/PMC13721315?pdf=render",
  "pmcid": "PMC13721315",
  "pmid": "42958422",
  "published": "2026-08-03",
  "publisher": "BMC",
  "ris": "https://research.draconitic.com/paper/2b0eqfkmzh3eq.ris",
  "sources": [
    {
      "first_seen": "2026-11-01T00:20:47Z",
      "native_id": "MED:42958422",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/42958422"
    },
    {
      "first_seen": "2026-11-03T12:22:01Z",
      "native_id": "PMC13721315",
      "source": "pmc",
      "url": "https://doi.org/10.1186/s13256-026-06312-4"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "openalex:2740|Sarcoma Diagnosis and Treatment",
      "subfield": "general"
    }
  ],
  "title": "Tazemetostat combined PD-1 inhibitor achieving prolonged survival in primary proximal epithelioid sarcoma: a case report and review of the literature.",
  "title_text": "Tazemetostat combined PD-1 inhibitor achieving prolonged survival in primary proximal epithelioid sarcoma: a case report and review of the literature.",
  "type": "peer-reviewed",
  "updated_at": "2026-11-03T12:22:01Z",
  "url": "https://research.draconitic.com/paper/2b0eqfkmzh3eq",
  "venue": "Journal of medical case reports",
  "versions": []
}