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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Format: | Article |
Language: | English |
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Elsevier
2023-03-01
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Series: | Pediatric Hematology Oncology Journal |
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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. |
first_indexed | 2024-04-09T20:07:43Z |
format | Article |
id | doaj.art-895c96f3b771455fb7982ec2209a4616 |
institution | Directory Open Access Journal |
issn | 2468-1245 |
language | English |
last_indexed | 2024-04-09T20:07:43Z |
publishDate | 2023-03-01 |
publisher | Elsevier |
record_format | Article |
series | Pediatric Hematology Oncology Journal |
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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