LG-ESSs and HG-ESSs: underlying molecular alterations and potential therapeutic strategies

Endometrial stromal tumors (ESTs) include endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated uterine sarcoma (UUS). Since these are rare tumor types, there is an unmet clinical need for the systemati...

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Published inJournal of Zhejiang University. B. Science Vol. 22; no. 8; pp. 633 - 646
Main Authors Li, Chunhui, Wang, Chunhong
Format Journal Article
LanguageEnglish
Published Hangzhou Zhejiang University Press 01.07.2021
Springer Nature B.V
Quality Management Office,The Second Hospital of Jilin University,Changchun 130041,China%Department of Hematology and Oncology,The Second Hospital of Jilin University,Changchun 130041,China
Subjects
14-3-3 Proteins
B-cell lymphoma
Biomedical and Life Sciences
Biomedicine
Carcinogenesis
Cytogenetics
Endometrial Neoplasms - classification
Endometrial Neoplasms - genetics
Endometrial Neoplasms - therapy
Endometrial Stromal Tumors - genetics
Endometrium
Female
Gene Duplication
Humans
Lymphocytes B
Lymphoma
Oncogene Proteins, Fusion
Proteins
Proto-Oncogene Proteins
Repressor Proteins
Review
RNA-Binding Proteins
Sarcoma
Sarcoma, Endometrial Stromal - genetics
Signal Transduction
Tryptophan
Tryptophan 5-monooxygenase
Tumorigenesis
Tumors
Tyrosine
Tyrosine 3-monooxygenase
Uterus
Zinc finger proteins
低级别子宫内膜间质肉瘤(LG-ESS)
治疗
Low-grade endometrial stromal sarcoma (LG-ESS)
Molecular genetics
高级别子宫内膜间质肉瘤(HG-ESS)
分子遗传学
Therapeutics
High-grade endometrial stromal sarcoma (HG-ESS)
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Abstract Endometrial stromal tumors (ESTs) include endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated uterine sarcoma (UUS). Since these are rare tumor types, there is an unmet clinical need for the systematic therapy of advanced LG-ESS or HG-ESS. Cytogenetic and molecular advances in ESTs have shown that multiple recurrent gene fusions are present in a large proportion of LG-ESSs, and HG-ESSs are identified by the tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein epsilon ( YWHAE )-family with sequence similarity 22 ( FAM22 ) fusion. Recently, a group of ESSs harboring both zinc finger CCCH domain-containing protein 7B ( ZC3H7B )-B-cell lymphoma 6 corepressor ( BCOR ) fusion and internal tandem duplication (ITD) of the BCOR gene have been provisionally classified as HG-ESSs. In this review, we firstly describe current knowledge about the molecular characteristics of recurrent aberrant proteins and their roles in the tumorigenesis of LG-ESSs and HG-ESSs. Next, we summarize the possibly shared signal pathways in the tumorigenesis of LG-ESSs and HG-ESSs, and list potentially actionable targets. Finally, based on the above discussion, we propose a few promising therapeutic strategies for LG-ESSs and HG-ESSs with recurrent gene alterations.
AbstractList Endometrial stromal tumors (ESTs) include endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated uterine sarcoma (UUS). Since these are rare tumor types, there is an unmet clinical need for the systematic therapy of advanced LG-ESS or HG-ESS. Cytogenetic and molecular advances in ESTs have shown that multiple recurrent gene fusions are present in a large proportion of LG-ESSs, and HG-ESSs are identified by the tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein epsilon (YWHAE)-family with sequence similarity 22 (FAM22) fusion. Recently, a group of ESSs harboring both zinc finger CCCH domain-containing protein 7B (ZC3H7B)-B-cell lymphoma 6 corepressor(BCOR) fusion and internal tandem duplication (ITD) of the BCOR gene have been provisionally classified as HG-ESSs. In this review, we firstly describe current knowledge about the molecular characteristics of recurrent aberrant proteins and their roles in the tumorigenesis of LG-ESSs and HG-ESSs. Next, we summarize the possibly shared signal pathways in the tumorigenesis of LG-ESSs and HG-ESSs, and list potentially actionable targets. Finally, based on the above discussion, we propose a few promising therapeutic strategies for LG-ESSs and HG-ESSs with recurrent gene alterations.Endometrial stromal tumors (ESTs) include endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated uterine sarcoma (UUS). Since these are rare tumor types, there is an unmet clinical need for the systematic therapy of advanced LG-ESS or HG-ESS. Cytogenetic and molecular advances in ESTs have shown that multiple recurrent gene fusions are present in a large proportion of LG-ESSs, and HG-ESSs are identified by the tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein epsilon (YWHAE)-family with sequence similarity 22 (FAM22) fusion. Recently, a group of ESSs harboring both zinc finger CCCH domain-containing protein 7B (ZC3H7B)-B-cell lymphoma 6 corepressor(BCOR) fusion and internal tandem duplication (ITD) of the BCOR gene have been provisionally classified as HG-ESSs. In this review, we firstly describe current knowledge about the molecular characteristics of recurrent aberrant proteins and their roles in the tumorigenesis of LG-ESSs and HG-ESSs. Next, we summarize the possibly shared signal pathways in the tumorigenesis of LG-ESSs and HG-ESSs, and list potentially actionable targets. Finally, based on the above discussion, we propose a few promising therapeutic strategies for LG-ESSs and HG-ESSs with recurrent gene alterations.
Endometrial stromal tumors (ESTs) include endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated uterine sarcoma (UUS). Since these are rare tumor types, there is an unmet clinical need for the systematic therapy of advanced LG-ESS or HG-ESS. Cytogenetic and molecular advances in ESTs have shown that multiple recurrent gene fusions are present in a large proportion of LG-ESSs, and HG-ESSs are identified by the tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein epsilon ( ) family with sequence similarity 22 ( ) fusion. Recently, a group of ESSs harboring both zinc finger CCCH domain-containing protein 7B ( )-B-cell lymphoma 6 corepressor( ) fusion and internal tandem duplication (ITD) of the gene have been provisionally classified as HG-ESSs. In this review, we firstly describe current knowledge about the molecular characteristics of recurrent aberrant proteins and their roles in the tumorigenesis of LG-ESSs and HG-ESSs. Next, we summarize the possibly shared signal pathways in the tumorigenesis of LG-ESSs and HG-ESSs, and list potentially actionable targets. Finally, based on the above discussion, we propose a few promising therapeutic strategies for LG-ESSs and HG-ESSs with recurrent gene alterations.
Endometrial stromal tumors (ESTs) include endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated uterine sarcoma (UUS). Since these are rare tumor types, there is an unmet clinical need for the systematic therapy of advanced LG-ESS or HG-ESS. Cytogenetic and molecular advances in ESTs have shown that multiple recurrent gene fusions are present in a large proportion of LG-ESSs, and HG-ESSs are identified by the tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein epsilon (YWHAE)-family with sequence similarity 22 (FAM22) fusion. Recently, a group of ESSs harboring both zinc finger CCCH domain-containing protein 7B (ZC3H7B)-B-cell lymphoma 6 corepressor (BCOR) fusion and internal tandem duplication (ITD) of the BCOR gene have been provisionally classified as HG-ESSs. In this review, we firstly describe current knowledge about the molecular characteristics of recurrent aberrant proteins and their roles in the tumorigenesis of LG-ESSs and HG-ESSs. Next, we summarize the possibly shared signal pathways in the tumorigenesis of LG-ESSs and HG-ESSs, and list potentially actionable targets. Finally, based on the above discussion, we propose a few promising therapeutic strategies for LG-ESSs and HG-ESSs with recurrent gene alterations.
Endometrial stromal tumors (ESTs) include endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated uterine sarcoma (UUS). Since these are rare tumor types, there is an unmet clinical need for the systematic therapy of advanced LG-ESS or HG-ESS. Cytogenetic and molecular advances in ESTs have shown that multiple recurrent gene fusions are present in a large proportion of LG-ESSs, and HG-ESSs are identified by the tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein epsilon ( YWHAE ) - family with sequence similarity 22 ( FAM22 ) fusion. Recently, a group of ESSs harboring both zinc finger CCCH domain-containing protein 7B ( ZC3H7B )-B-cell lymphoma 6 corepressor( BCOR ) fusion and internal tandem duplication (ITD) of the BCOR gene have been provisionally classified as HG-ESSs. In this review, we firstly describe current knowledge about the molecular characteristics of recurrent aberrant proteins and their roles in the tumorigenesis of LG-ESSs and HG-ESSs. Next, we summarize the possibly shared signal pathways in the tumorigenesis of LG-ESSs and HG-ESSs, and list potentially actionable targets. Finally, based on the above discussion, we propose a few promising therapeutic strategies for LG-ESSs and HG-ESSs with recurrent gene alterations.
Endometrial stromal tumors (ESTs) include endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated uterine sarcoma (UUS). Since these are rare tumor types, there is an unmet clinical need for the systematic therapy of advanced LG-ESS or HG-ESS. Cytogenetic and molecular advances in ESTs have shown that multiple recurrent gene fusions are present in a large proportion of LG-ESSs, and HG-ESSs are identified by the tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein epsilon ( YWHAE )-family with sequence similarity 22 ( FAM22 ) fusion. Recently, a group of ESSs harboring both zinc finger CCCH domain-containing protein 7B ( ZC3H7B )-B-cell lymphoma 6 corepressor ( BCOR ) fusion and internal tandem duplication (ITD) of the BCOR gene have been provisionally classified as HG-ESSs. In this review, we firstly describe current knowledge about the molecular characteristics of recurrent aberrant proteins and their roles in the tumorigenesis of LG-ESSs and HG-ESSs. Next, we summarize the possibly shared signal pathways in the tumorigenesis of LG-ESSs and HG-ESSs, and list potentially actionable targets. Finally, based on the above discussion, we propose a few promising therapeutic strategies for LG-ESSs and HG-ESSs with recurrent gene alterations.
Author Wang, Chunhong
Li, Chunhui
AuthorAffiliation Quality Management Office,The Second Hospital of Jilin University,Changchun 130041,China%Department of Hematology and Oncology,The Second Hospital of Jilin University,Changchun 130041,China
AuthorAffiliation_xml – name: Quality Management Office,The Second Hospital of Jilin University,Changchun 130041,China%Department of Hematology and Oncology,The Second Hospital of Jilin University,Changchun 130041,China
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  surname: Li
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  surname: Wang
  fullname: Wang, Chunhong
  email: wang_ch@jlu.edu.cn
  organization: Department of Hematology and Oncology, The Second Hospital of Jilin University
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DocumentTitle_FL 低级别和高级别子宫内膜间质肉瘤的分子改变与治疗策略
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Issue 8
Keywords 低级别子宫内膜间质肉瘤(LG-ESS)
治疗
Low-grade endometrial stromal sarcoma (LG-ESS)
Molecular genetics
高级别子宫内膜间质肉瘤(HG-ESS)
分子遗传学
Therapeutics
High-grade endometrial stromal sarcoma (HG-ESS)
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  year: 2021
  text: 2021-07-01
  day: 01
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– name: Editorial Office of Journal of Zhejiang University-SCIENCE, 148 Tianmushan Road, Hangzhou 310028, China
PublicationSubtitle Biomedicine & Biotechnology
PublicationTitle Journal of Zhejiang University. B. Science
PublicationTitleAbbrev J. Zhejiang Univ. Sci. B
PublicationTitleAlternate J Zhejiang Univ Sci B
PublicationTitle_FL Journal of Zhejiang University Science B：Biomedicine & Biotechnology
PublicationYear 2021
Publisher Zhejiang University Press
Springer Nature B.V
Quality Management Office,The Second Hospital of Jilin University,Changchun 130041,China%Department of Hematology and Oncology,The Second Hospital of Jilin University,Changchun 130041,China
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Snippet Endometrial stromal tumors (ESTs) include endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal...
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SubjectTerms 14-3-3 Proteins
B-cell lymphoma
Biomedical and Life Sciences
Biomedicine
Carcinogenesis
Cytogenetics
Endometrial Neoplasms - classification
Endometrial Neoplasms - genetics
Endometrial Neoplasms - therapy
Endometrial Stromal Tumors - genetics
Endometrium
Female
Gene Duplication
Humans
Lymphocytes B
Lymphoma
Oncogene Proteins, Fusion
Proteins
Proto-Oncogene Proteins
Repressor Proteins
Review
RNA-Binding Proteins
Sarcoma
Sarcoma, Endometrial Stromal - genetics
Signal Transduction
Tryptophan
Tryptophan 5-monooxygenase
Tumorigenesis
Tumors
Tyrosine
Tyrosine 3-monooxygenase
Uterus
Zinc finger proteins
Title LG-ESSs and HG-ESSs: underlying molecular alterations and potential therapeutic strategies
URI https://link.springer.com/article/10.1631/jzus.B2000797
https://www.ncbi.nlm.nih.gov/pubmed/34414699
https://www.proquest.com/docview/2565812872
https://www.proquest.com/docview/2563424503
https://d.wanfangdata.com.cn/periodical/zjdxxbb-e202108003
https://pubmed.ncbi.nlm.nih.gov/PMC8377580
Volume 22
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