Chromoanagenesis in Osteosarcoma

Chromoanagenesis is a catastrophic genomic phenomenon involving sudden, extensive rearrangements within one or a few cell cycles. In osteosarcoma, the most prevalent malignant bone tumor in children and adolescents, these events dramatically alter the genomic landscape, frequently disrupting key tum...

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Main Authors: Guozhuang Li, Nan Wu, Jen Ghabrial, Victoria Stinnett, Melanie Klausner, Laura Morsberger, Patty Long, Ezra Baraban, John M. Gross, Ying S. Zou
Format: Article
Language:English
Published: MDPI AG 2025-06-01
Series:Biomolecules
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Online Access:https://www.mdpi.com/2218-273X/15/6/833
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author Guozhuang Li
Nan Wu
Jen Ghabrial
Victoria Stinnett
Melanie Klausner
Laura Morsberger
Patty Long
Ezra Baraban
John M. Gross
Ying S. Zou
author_facet Guozhuang Li
Nan Wu
Jen Ghabrial
Victoria Stinnett
Melanie Klausner
Laura Morsberger
Patty Long
Ezra Baraban
John M. Gross
Ying S. Zou
author_sort Guozhuang Li
collection DOAJ
description Chromoanagenesis is a catastrophic genomic phenomenon involving sudden, extensive rearrangements within one or a few cell cycles. In osteosarcoma, the most prevalent malignant bone tumor in children and adolescents, these events dramatically alter the genomic landscape, frequently disrupting key tumor suppressor genes like <i>TP53</i> and <i>RB1</i>, amplifying oncogene expression, and propelling tumor progression and evolution. This review elucidates how key chromoanagenic mechanisms, such as chromothripsis and chromoanasynthesis, arise from replication stress and impaired DNA repair pathways, ultimately contributing to genomic instability in osteosarcoma. Chromothripsis features prominently in osteosarcoma, occurring in up to 62% of tumor regions and driving intratumoral heterogeneity through persistent genomic crises. Next-generation sequencing, optical genome mapping, and emerging technologies like single-cell sequencing empower researchers to detect and characterize these complex structural variants, demonstrating how a single catastrophic event can profoundly influence osteosarcoma progression over time. While targeted therapies for osteosarcoma have proven elusive, innovative strategies harnessing comprehensive genomic profiling and patient-derived preclinical models hold promise for uncovering tumor-specific vulnerabilities tied to chromoanagenesis. Ultimately, unraveling how these rapid, large-scale rearrangements fuel osteosarcoma’s aggressive nature will not only refine disease classification and prognosis but also pave the way for novel therapeutic approaches to enhance patient outcomes.
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spelling doaj-art-9febbfc9aeaf4967b2ec9ff5a89c1d232025-08-20T03:27:05ZengMDPI AGBiomolecules2218-273X2025-06-0115683310.3390/biom15060833Chromoanagenesis in OsteosarcomaGuozhuang Li0Nan Wu1Jen Ghabrial2Victoria Stinnett3Melanie Klausner4Laura Morsberger5Patty Long6Ezra Baraban7John M. Gross8Ying S. Zou9State Key Laboratory of Complex Severe and Rare Diseases, Department of Orthopedic Surgery, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, ChinaState Key Laboratory of Complex Severe and Rare Diseases, Department of Orthopedic Surgery, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, ChinaDepartment of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USADepartment of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USADepartment of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USADepartment of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USADepartment of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USADepartment of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USADepartment of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USADepartment of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USAChromoanagenesis is a catastrophic genomic phenomenon involving sudden, extensive rearrangements within one or a few cell cycles. In osteosarcoma, the most prevalent malignant bone tumor in children and adolescents, these events dramatically alter the genomic landscape, frequently disrupting key tumor suppressor genes like <i>TP53</i> and <i>RB1</i>, amplifying oncogene expression, and propelling tumor progression and evolution. This review elucidates how key chromoanagenic mechanisms, such as chromothripsis and chromoanasynthesis, arise from replication stress and impaired DNA repair pathways, ultimately contributing to genomic instability in osteosarcoma. Chromothripsis features prominently in osteosarcoma, occurring in up to 62% of tumor regions and driving intratumoral heterogeneity through persistent genomic crises. Next-generation sequencing, optical genome mapping, and emerging technologies like single-cell sequencing empower researchers to detect and characterize these complex structural variants, demonstrating how a single catastrophic event can profoundly influence osteosarcoma progression over time. While targeted therapies for osteosarcoma have proven elusive, innovative strategies harnessing comprehensive genomic profiling and patient-derived preclinical models hold promise for uncovering tumor-specific vulnerabilities tied to chromoanagenesis. Ultimately, unraveling how these rapid, large-scale rearrangements fuel osteosarcoma’s aggressive nature will not only refine disease classification and prognosis but also pave the way for novel therapeutic approaches to enhance patient outcomes.https://www.mdpi.com/2218-273X/15/6/833chromoanagenesisosteosarcomastructural variantchromothripsischromoanasynthesiscopy number variant
spellingShingle Guozhuang Li
Nan Wu
Jen Ghabrial
Victoria Stinnett
Melanie Klausner
Laura Morsberger
Patty Long
Ezra Baraban
John M. Gross
Ying S. Zou
Chromoanagenesis in Osteosarcoma
Biomolecules
chromoanagenesis
osteosarcoma
structural variant
chromothripsis
chromoanasynthesis
copy number variant
title Chromoanagenesis in Osteosarcoma
title_full Chromoanagenesis in Osteosarcoma
title_fullStr Chromoanagenesis in Osteosarcoma
title_full_unstemmed Chromoanagenesis in Osteosarcoma
title_short Chromoanagenesis in Osteosarcoma
title_sort chromoanagenesis in osteosarcoma
topic chromoanagenesis
osteosarcoma
structural variant
chromothripsis
chromoanasynthesis
copy number variant
url https://www.mdpi.com/2218-273X/15/6/833
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