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Vol 73, No 3 (2023)
Review paper
Published online: 2023-05-11
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Epithelioid sarcoma

Anna M. Czarnecka1
DOI: 10.5603/NJO.a2023.0021
·
Nowotwory. Journal of Oncology 2023;73(3):154-161.
Affiliations
  1. Department of Soft Tissue, Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland

open access

Vol 73, No 3 (2023)
Rare neoplasms
Published online: 2023-05-11

Abstract

Epithelioid sarcoma (ES) is a very rare sarcoma characterized by loss of INI1. Enzinger first described ES in 1970, but the histopathologic differential diagnosis of ES remains challenging. There are two ES subtypes, the classical type with spindle epithelioid to the central pseudogranulomatous cells, and the proximal type, which is predominantly composed of epithelioid and rhabdoid cells. ES symptoms and signs are not specific and depend on tumor localization. The only treatment for ES is radical excision with a microscope-radical margin. In general, the best treatment for ES in extremes is radical resection with a wide margin or amputation with or without lymph node dissection. Surgery may be followed by adjuvant chemotherapy and/or radiation therapy. Referral of patients with ES to a sarcoma center that offers hypofractionation RT trials and multidisciplinary clinical trials should be considered upfront. Neoadjuvant chemotherapy with ifosfamide and doxorubicin with / or without radiation therapy must be used after a multidisciplinary team discussion. On 23 January 2020, the US Food and Drug Administration (FDA) first approved tazemetostat – an inhibitor of zeste homolog 2 enhancer – therapy for metastatic ES or locally advanced ES not eligible for radical resection.

Abstract

Epithelioid sarcoma (ES) is a very rare sarcoma characterized by loss of INI1. Enzinger first described ES in 1970, but the histopathologic differential diagnosis of ES remains challenging. There are two ES subtypes, the classical type with spindle epithelioid to the central pseudogranulomatous cells, and the proximal type, which is predominantly composed of epithelioid and rhabdoid cells. ES symptoms and signs are not specific and depend on tumor localization. The only treatment for ES is radical excision with a microscope-radical margin. In general, the best treatment for ES in extremes is radical resection with a wide margin or amputation with or without lymph node dissection. Surgery may be followed by adjuvant chemotherapy and/or radiation therapy. Referral of patients with ES to a sarcoma center that offers hypofractionation RT trials and multidisciplinary clinical trials should be considered upfront. Neoadjuvant chemotherapy with ifosfamide and doxorubicin with / or without radiation therapy must be used after a multidisciplinary team discussion. On 23 January 2020, the US Food and Drug Administration (FDA) first approved tazemetostat – an inhibitor of zeste homolog 2 enhancer – therapy for metastatic ES or locally advanced ES not eligible for radical resection.

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Keywords

sarcoma; epithelioid sarcoma; Enzinger; tazemetostat

About this article
Title

Epithelioid sarcoma

Journal

Nowotwory. Journal of Oncology

Issue

Vol 73, No 3 (2023)

Article type

Review paper

Pages

154-161

Published online

2023-05-11

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1312

Article views/downloads

420

DOI

10.5603/NJO.a2023.0021

Bibliographic record

Nowotwory. Journal of Oncology 2023;73(3):154-161.

Keywords

sarcoma
epithelioid sarcoma
Enzinger
tazemetostat

Authors

Anna M. Czarnecka

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