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Czarnecka AM, Sobczuk P, Kostrzanowski M, et al. Epithelioid Sarcoma-From Genetics to Clinical Practice. Cancers (Basel). 2020;12(8)doi: 10.3390/cancers12082112.
Czarnecka, A. M., Sobczuk, P., Kostrzanowski, M., Spalek, M., Chojnacka, M., Szumera-Cieckiewicz, A., & Rutkowski, P. (2020). Epithelioid Sarcoma-From Genetics to Clinical Practice. Cancers, 12(8), . https://doi.org/10.3390/cancers12082112
Czarnecka, Anna M, et al. "Epithelioid Sarcoma-From Genetics to Clinical Practice." Cancers vol. 12,8 (2020). doi: https://doi.org/10.3390/cancers12082112
Czarnecka AM, Sobczuk P, Kostrzanowski M, Spalek M, Chojnacka M, Szumera-Cieckiewicz A, Rutkowski P. Epithelioid Sarcoma-From Genetics to Clinical Practice. Cancers (Basel). 2020 Jul 29;12(8). doi: 10.3390/cancers12082112. PMID: 32751241; PMCID: PMC7463637.
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Cancers (Basel). 2020 Jul 29;12(8). doi: 10.3390/cancers12082112.

Epithelioid Sarcoma-From Genetics to Clinical Practice.

Cancers

Anna M Czarnecka, Pawel Sobczuk, Michal Kostrzanowski, Mateusz Spalek, Marzanna Chojnacka, Anna Szumera-Cieckiewicz, Piotr Rutkowski

Affiliations

  1. Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland.
  2. Department of Experimental Pharmacology, Mossakowski Medical Research Centre, Polish Academy of Sciences, 02-106 Warsaw, Poland.
  3. Department of Experimental and Clinical Physiology, Laboratory of Centre for Preclinical Research, Medical University of Warsaw, 02-097 Warsaw, Poland.
  4. Faculty of Medicine, Medical University of Warsaw, 02-091 Warsaw, Poland.
  5. Department of Radiotherapy, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland.
  6. Department of Pathology and Laboratory Diagnostics, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland.
  7. Department of Diagnostic Hematology, Institute of Hematology and Transfusion Medicine, 02-776 Warsaw, Poland.

PMID: 32751241 PMCID: PMC7463637 DOI: 10.3390/cancers12082112

Abstract

Epithelioid sarcoma is a mesenchymal soft tissue sarcoma often arising in the extremities, usually in young adults with a pick of incidence at 35 years of age. Epithelioid sarcoma (ES) is characterized by the loss of SMARCB1/INI1 (integrase interactor 1) or other proteins of the SWI/SNF complex. Two distinct types, proximal and distal, with varying biology and treatment outcomes, are distinguished. ES is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. An optimal long-term management strategy is still to be defined. The best treatment of localized ES is wide surgical resection. Neo-adjuvant or adjuvant radiotherapy may be recommended, as it reduces the local recurrence rate. Sentinel lymph node biopsy should be considered in ES patients. Patients with metastatic ES have a poor prognosis with an expected median overall survival of about a year. Doxorubicin-based regimens are recommended for advanced ES. Tazemetostat, an EZH2 methyltransferase, has shown promising results in ES patients. Novel therapies, including immunotherapy, are still needed.

Keywords: EZH2; SMARCB1; chemotherapy; epithelioid sarcoma; radiotherapy; surgery; tazemetostat

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