Ewing sarcoma genomics and recent therapeutic advancements

Ewing sarcomas are highly heterogenous mesenchymal tumors that develop in bone or soft tissue and primarily affect children, teenagers, and young adults. After osteosarcoma, it is the second most common malignant bone sarcoma. Ewing sarcoma, metastatic and relapsed, typically have a poor prognosis a...

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Main Authors: Manisha Chavan, Sandesh Dhakal, Ajay Singh, Vikramaditya Rai, Sanjiya Arora, Mohitha C Mallipeddi, Arghadip Das
Format: Article
Language:English
Published: Elsevier 2023-03-01
Series:Pediatric Hematology Oncology Journal
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2468124523000086
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author Manisha Chavan
Sandesh Dhakal
Ajay Singh
Vikramaditya Rai
Sanjiya Arora
Mohitha C Mallipeddi
Arghadip Das
author_facet Manisha Chavan
Sandesh Dhakal
Ajay Singh
Vikramaditya Rai
Sanjiya Arora
Mohitha C Mallipeddi
Arghadip Das
author_sort Manisha Chavan
collection DOAJ
description Ewing sarcomas are highly heterogenous mesenchymal tumors that develop in bone or soft tissue and primarily affect children, teenagers, and young adults. After osteosarcoma, it is the second most common malignant bone sarcoma. Ewing sarcoma, metastatic and relapsed, typically have a poor prognosis and recurrences are frequent, leading to high rates of morbidity and mortality. The ongoing inability to increase overall survival for patients with ewing sarcoma emphasizes the critical requirement for the quick translation of emerging therapy approaches. Targeting the EWR1/FLI1 fusion protein, which is the primary genetic anomaly and master regulator of ewing sarcoma and found in 80–90% of instances of ewing tumors, is the most crucial objective. This review offers new insights into the genomics and proteomics of ewing sarcoma signaling and how it influences the tumor microenvironment and disease progression. It also elucidates how recent technological advancements have explained some of the underlying oncogenic characteristics of ewing sarcoma. The current review examined existing and potential experimental therapies that, by enhancing patient survival and quality of life, target multiple signaling pathways involved in the progression of ewing sarcoma. These therapies may one day replace current regimens as the new standard of care for patients.
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spelling doaj.art-895c96f3b771455fb7982ec2209a46162023-04-01T08:50:52ZengElsevierPediatric Hematology Oncology Journal2468-12452023-03-01815065Ewing sarcoma genomics and recent therapeutic advancementsManisha Chavan0Sandesh Dhakal1Ajay Singh2Vikramaditya Rai3Sanjiya Arora4Mohitha C Mallipeddi5Arghadip Das6Department of Medicine, Kakatiya Medical College, Rangam Peta, Warangal, Telangana, IndiaDepartment of Medicine, College of Medical Sciences, Bharatpur, NepalDepartment of Medicine, Sri Ram Murti Smarak Institute of Medical Sciences, Bareilly, Uttar Pradesh, IndiaDepartment of Orthopedics, Dr. Rajindra Prasad Government Medical College, Tanda at Kangra, Himachal Pradesh, India; Corresponding author. Department of Orthopedics, Dr. Rajindra Prasad Government Medical College, Tanda, Kangra, Himachal Pradesh, India.Department of Medicine, Sub-Divisional Hospital cum Civil Hospital, Ratia, Fatehabad, Haryana, IndiaDepartment of Medicine, Sri Padmavathi Medical College, Tirupathi, Andhra Pradesh, IndiaDepartment of Medicine, Nilratan Sircar Medical College and Hospital, Kolkata, West Bengal, IndiaEwing sarcomas are highly heterogenous mesenchymal tumors that develop in bone or soft tissue and primarily affect children, teenagers, and young adults. After osteosarcoma, it is the second most common malignant bone sarcoma. Ewing sarcoma, metastatic and relapsed, typically have a poor prognosis and recurrences are frequent, leading to high rates of morbidity and mortality. The ongoing inability to increase overall survival for patients with ewing sarcoma emphasizes the critical requirement for the quick translation of emerging therapy approaches. Targeting the EWR1/FLI1 fusion protein, which is the primary genetic anomaly and master regulator of ewing sarcoma and found in 80–90% of instances of ewing tumors, is the most crucial objective. This review offers new insights into the genomics and proteomics of ewing sarcoma signaling and how it influences the tumor microenvironment and disease progression. It also elucidates how recent technological advancements have explained some of the underlying oncogenic characteristics of ewing sarcoma. The current review examined existing and potential experimental therapies that, by enhancing patient survival and quality of life, target multiple signaling pathways involved in the progression of ewing sarcoma. These therapies may one day replace current regimens as the new standard of care for patients.http://www.sciencedirect.com/science/article/pii/S2468124523000086Ewing sarcomaGenomicsTumorImmunotherapyFusion protein
spellingShingle Manisha Chavan
Sandesh Dhakal
Ajay Singh
Vikramaditya Rai
Sanjiya Arora
Mohitha C Mallipeddi
Arghadip Das
Ewing sarcoma genomics and recent therapeutic advancements
Pediatric Hematology Oncology Journal
Ewing sarcoma
Genomics
Tumor
Immunotherapy
Fusion protein
title Ewing sarcoma genomics and recent therapeutic advancements
title_full Ewing sarcoma genomics and recent therapeutic advancements
title_fullStr Ewing sarcoma genomics and recent therapeutic advancements
title_full_unstemmed Ewing sarcoma genomics and recent therapeutic advancements
title_short Ewing sarcoma genomics and recent therapeutic advancements
title_sort ewing sarcoma genomics and recent therapeutic advancements
topic Ewing sarcoma
Genomics
Tumor
Immunotherapy
Fusion protein
url http://www.sciencedirect.com/science/article/pii/S2468124523000086
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