Epithelioid Sarcoma-From Genetics to Clinical Practice

被引：44

作者：

Czarnecka, Anna M. ^{[1
,2
]}

Sobczuk, Pawel ^{[1
,3
]}

Kostrzanowski, Michal ^{[1
,4
]}

Spalek, Mateusz ^{[1
]}

Chojnacka, Marzanna ^{[5
]}

Szumera-Cieckiewicz, Anna ^{[6
,7
]}

Rutkowski, Piotr ^{[1
]}

机构：

[1] Maria Sklodowska Curie Natl Res Inst Oncol, Dept Soft Tissue Bone Sarcoma & Melanoma, PL-02781 Warsaw, Poland

[2] Polish Acad Sci, Mossakowski Med Res Ctr, Dept Expt Pharmacol, PL-02106 Warsaw, Poland

[3] Med Univ Warsaw, Lab Ctr Preclin Res, Dept Expt & Clin Physiol, PL-02097 Warsaw, Poland

[4] Med Univ Warsaw, Fac Med, PL-02091 Warsaw, Poland

[5] Maria Sklodowska Curie Natl Res Inst Oncol, Dept Radiotherapy, PL-02781 Warsaw, Poland

[6] Maria Sklodowska Curie Natl Res Inst Oncol, Dept Pathol & Lab Diagnost, PL-02781 Warsaw, Poland

[7] Inst Hematol & Transfus Med, Dept Diagnost Hematol, PL-02776 Warsaw, Poland

来源：

CANCERS | 2020年 / 12卷 / 08期

关键词：

epithelioid sarcoma; SMARCB1; EZH2; surgery; radiotherapy; chemotherapy; tazemetostat; SOFT-TISSUE SARCOMA; LYMPH-NODE BIOPSY; PROXIMAL-TYPE; PROGNOSTIC-SIGNIFICANCE; ONCOLOGY GROUP; GROWTH-FACTOR; EXPRESSION; FEATURES; RARE; EXPERIENCE;

D O I：

10.3390/cancers12082112

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

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.

引用

页码：1 / 19

页数：19

共 117 条

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