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Learning objectives

By the end of this session participants will be better able to:

  • Recognize early signs and symptoms of epithelioid sarcoma to improve timely and accurate diagnosis.
  • Interpret INI1 loss and histopathologic features to achieve an accurate and timely diagnosis of epithelioid sarcoma.
  • Evaluate and apply best practices in treatment management of epithelioid sarcoma, including first-line therapy selection and sequencing.

References

  1. Armah HB, Parwani AV. Epithelioid sarcoma. Arch Pathol Lab Med. 2009;133(5):814-819.
  2. Carter CS, Patel RM. Cutaneous soft tissue tumors: Diagnostically disorienting epithelioid tumors that are not epithelial, and other perplexing mesenchymal lesions. Mod Pathol. 2020;33:66-82.
  3. Czarnecka AM. Epithelioid sarcoma. Nowotwory. Journal of Oncology. 2023;73:154–161.
  4. Czarnecka AM, Sobczuk P, Kostrzanowski M, et al. Epithelioid sarcoma—from genetics to clinical practice. Cancers (Basel). 2020;12(8):2112.
  5. Del Savio E, Maestro R. Beyond SMARCB1 Loss: Recent Insights into the Pathobiology of Epithelioid Sarcoma. Cells.2022;11:2626.
  6. Frezza AM, Jones RL, Lo Vullo S, et al. Anthracycline, Gemcitabine, and Pazopanib in Epithelioid Sarcoma: A Multi-institutional Case Series. JAMA Oncol. 2018;4:e180219.
  7. Fuchs JR, et al. Treatment of Advanced Epithelioid Sarcoma with Combination Tazemetostat and Pembrolizumab, Connective Tissue Oncology Society 2025.
  8. Gounder MM, et al. Real-world outcomes of patients with locally advanced or metastatic epithelioid sarcoma. Cancer.2021;127(8):1311-1317.
  9. Gronchi A, et al. Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2021;32:1348-1365.
  10. Grünewald TGP, et al. Translational Aspects of Epithelioid Sarcoma: Current Consensus. Clin Cancer Res.2024;30:1079-1092.
  11. Guillou L, et al. "Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series. Am J Surg Pathol. 1997;21:130-146.
  12. Kohashi K, Oda Y. Oncogenic roles of SMARCB1/INI1 and its deficient tumors. Cancer Sci. 2017;108:547-552.
  13. Kreiger PA, et al. Loss of INI1 expression defines a unique subset of pediatric undifferentiated soft tissue sarcomas. Mod Pathol. 2009;22:142-150.
  14. Liu J, Fu Y, Wang B, et al. Eribulin plus anlotinib in advanced soft tissue sarcoma (ERAS): Updates on efficacy and biomarkers. J Clin Oncol. 2025;43(16_suppl):11502.
  15. Liu J, Tan Z, Guo X, et al. Safety and antitumor activity of 177Lu-catelase: A preliminary clinical study in soft tissue sarcoma. J Clin Oncol. 2025;43(16_suppl):11563.
  16. Mavroidis et al. 88P: Real-world evidence of the efficacy of ipilimumab plus nivolumab in patients with epithelioid sarcoma. ESMO Open. 2024;9:2059-7029.
  17. MedAll Survey Results. March 2025.
  18. Miettinen M, et al. Epithelioid sarcoma: An immunohistochemical analysis of 112 classical and variant cases and a discussion of the differential diagnosis. Hum Pathol. 1999;30:934–942.
  19. NCCN Guidelines. Soft tissue sarcoma. Version 3.2026 - March 12, 2026. Available at: NCCN.org (accessed 27 March 2026).
  20. Sakamoto A, Jono O, Hirahashi M, et al. Epithelioid sarcoma with muscle metastasis detected by positron emission tomography. World J Surg Oncol. 2008;6:84.
  21. Shakya S, Banneyake EL, Cholekho S, Singh J, Zhou X. Soft tissue sarcoma: clinical recognition and approach to the loneliest cancer. Explor Musculoskeletal Dis. 2024;2:56–68.
  22. Wang J, et al. Response to immunotherapy in a patient with advanced epithelioid sarcoma of adrenal gland: A case report. Experimental and Therapeutic Medicine. 2022;24:659.
  23. Wedekind MF, Akshintala S, Widemann BC, et al. Phase 1/2 study of tiragolumab and atezolizumab in patients with relapsed or refractory SMARCB1- or SMARCA4-deficient tumors: PEPN2121. J Clin Oncol.2025;43(16_suppl):TPS2695.
  24. Zhang X, Pan Q, Zhang P, et al. Multicenter phase II study of anlotinib and toripalimab in patients with advanced soft tissue sarcoma (STS) and bone sarcoma (BS). J Clin Oncol. 2025;43(16_suppl):11556.
  25. Zhou Y, Niu X, Jiang Y, et al. Anlotinib in combination with epirubicin followed by maintenance anlotinib versus placebo plus epirubicin as first-line treatment for advanced soft tissue sarcoma (STS): A randomized, double-blind, parallel-controlled, phase III study. J Clin Oncol. 2025;43(16_suppl):11501.
Specialities: Oncology
Estimated time: 15 minutes
Credit types: AMA
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This program is supported by an independent education grant from Ipsen. This online education program has been designed for healthcare professionals in the US. The content is not intended for healthcare professionals in any other country.

Faculty

Seth M. Pollack, MD is an oncologist and physician-scientist specializing in sarcoma and cancer immunotherapy. As the Director of the Sarcoma Program at Northwestern University, he focuses on developing new treatments for soft tissue sarcomas. His research primarily addresses why some tumors do not respond to traditional immunotherapy and investigates methods to improve the effectiveness of T-cell therapies in these "immune-cold" cancers.

Dr. Pollack leads several clinical trials, including first-in-human studies that test cytokine-based strategies and engineered T cells. He currently serves as the national chair for a Phase III trial evaluating immunotherapy combinations for advanced sarcoma. In addition to his clinical work, he contributes to the development of national treatment guidelines and serves on the advisory boards of several major sarcoma research foundations.

Hannah Abrams, MD is a hematology/oncology fellow at the Fred Hutch Cancer Center and a contributor to the medical education podcast The Curious Clinicians. Her clinical practice focuses on the management of hematologic malignancies, with a professional interest in the clinical reasoning and evidence-based foundations of modern oncology. Her recent health services research examines the socioeconomic and logistical barriers to advanced cellular therapies.

This continuing education activity is active starting April 24rd 2026 and will expire on June 5th 2026.