ArticleESMO open2022
Systemic treatment of advanced clear cell sarcoma: results from a retrospective international series from the World Sarcoma Network.
Article in ESMO open, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
19 citing papers in PubMed, 33 citations in OpenAlex.
- IMMUNOSARC II Master Trial: Phase II Study of Sunitinib and Nivolumab in Clear Cell Sarcoma Cohort.Cancer communications (London, England) · 2026Trial
- Revisiting the Immune Frontier in Soft Tissue Sarcomas.Current oncology reports · 2026Review
- FDG PET/CT as the Decisive Modality in Distinguishing Clear Cell Sarcoma from Melanoma When Immunohistochemistry Overlaps: A Case Report.World journal of nuclear medicine · 2026Article
- Clear Cell and Histiocytic/Dendritic Cell Sarcomas: Clinical Outcomes, Molecular Features, and Diagnostic Pitfalls.Cancers · 2026Article
- Cadonilimab in the treatment of advanced metastatic soft tissue clear cell sarcoma achieved complete remission: a case report.Frontiers in oncology · 2026Article
- The Role of Targeted Therapy and Immunotherapy in Metastatic GNET/Clear Cell Sarcoma (CCS) of the Gastrointestinal Tract: A Case Report.Current issues in molecular biology · 2025Article
- Clear Cell Sarcoma Incidence and Survival: A Surveillance, Epidemiology, and End Results (SEER) Database Analysis.Cureus · 2025Article
- Clinical characterization and therapeutic targeting of fusion genes in oncology.Future oncology (London, England) · 2025Review
- Establishing preclinical models for clear cell sarcoma of soft tissue.Frontiers in oncology · 2025Article
- Malignant melanoma: An important differential diagnosis for clear cell sarcoma of the gastrointestinal tract.World journal of clinical cases · 2024Article
- Prognostic factors in clear cell sarcoma: an analysis of soft tissue sarcoma in 43 cases.Journal of cancer research and clinical oncology · 2024Article
- Essential role of postoperative follow-up in the management of clear cell sarcoma.World journal of clinical cases · 2024Article
- Metastatic clear cell sarcoma of the pancreas: A rare case report.World journal of clinical cases · 2024Article
- Systemic Therapy in Advanced Pleomorphic Liposarcoma: a Comprehensive Review.Current treatment options in oncology · 2023Review
- Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma.Current oncology (Toronto, Ont.) · 2023Review
- Randomized Phase II Trial of Sunitinib or Cediranib in Alveolar Soft Part Sarcoma.Clinical cancer research : an official journal of the American Association for Cancer Research · 2023Article
- Case report: Robust response of metastatic clear cell sarcoma treated with cabozantinib and immunotherapy.Frontiers in pediatrics · 2022Article
- A Clear Cell Sarcoma Case: A Diagnostic and Treatment Challenge, with a Promising Response to Trabectedin.Case reports in oncologyArticle
- Challenges in the Management of Stage IV Clear Cell Sarcoma of Soft Tissue in Young Adults: Case Report and Review of the Literature.Case reports in oncologyArticle
Corrections and comments
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Authors and funding
18 authors at 12 institutions in 7 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundClear cell sarcoma (CCS) is a translocated aggressive malignancy with a high incidence of metastases and poor prognosis. There are few studies describing the activity of systemic therapy in CCS. We report a multi-institutional retrospective study of the outcomes of patients with advanced CCS treated with systemic therapy within the World Sarcoma Network (WSN). MATERIALS AND
methodsPatients with molecularly confirmed locally advanced or metastatic CCS treated with systemic therapy from June 1985 to May 2021 were included. Baseline demographic and treatment information, including response by Response Evaluation Criteria in Solid Tumours (RECIST) 1.1, was retrospectively collected by local investigators. Descriptive statistics were carried out.
resultsFifty-five patients from 10 institutions were included. At diagnosis, the median age was 30 (15-73) years and 24% (n = 13/55) had metastatic disease. The median age at diagnosis was 30 (15-73) years. Most primary tumours were at aponeurosis (n = 9/55, 16%) or non-aponeurosis limb sites (n = 17/55, 31%). The most common fusion was EWSR1-ATF1 (n = 24/55, 44%). The median number of systemic therapies was 1 (range 1-7). The best response rate was seen for patients treated with sunitinib (30%, n = 3/10), with a median progression-free survival of 4 [95% confidence interval (CI) 1-7] months. The median overall survival for patients with advanced/metastatic disease was 15 months (95% CI 3-27 months).
conclusionsSoft tissue sarcoma-type systemic therapies have limited benefit in advanced CCS and response rate was poor. International, multicentre prospective translational studies are required to identify new treatments for this ultra-rare subtype, and access to early clinical trial enrolment remains key for patients with CCS.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.