Contemporary preclinical mouse models for pediatric rhabdomyosarcoma: from bedside to bench to bedside
Background: Rhabdomyosarcoma (RMS) is the most common pediatric softtissue malignancy, characterized by high clinicalopathological and molecular heterogeneity. Preclinical in vivo models are essential for advancing our understanding of RMS oncobiology and developing novel treatment strategies. H...
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Format: | Artikel |
Sprache: | Englisch |
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Philipps-Universität Marburg
2024
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Zusammenfassung: | Background: Rhabdomyosarcoma (RMS) is the most common pediatric softtissue
malignancy, characterized by high clinicalopathological and molecular
heterogeneity. Preclinical in vivo models are essential for advancing our
understanding of RMS oncobiology and developing novel treatment strategies.
However, the diversity of scholarly data on preclinical RMS studies may challenge
scientists and clinicians. Hence, we performed a systematic literature survey of
contemporary RMS mouse models to characterize their phenotypes and assess
their translational relevance.
Methods: We identified papers published between 01/07/2018 and 01/07/2023
by searching PubMed and Web of Science databases.
Results: Out of 713 records screened, 118 studies (26.9%) were included in the
qualitative synthesis. Cell line-derived xenografts (CDX) were the most
commonly utilized (n = 75, 63.6%), followed by patient-derived xenografts
(PDX) and syngeneic models, each accounting for 11.9% (n = 14), and
genetically engineered mouse models (GEMM) (n = 7, 5.9%). Combinations of
different model categories were reported in 5.9% (n = 7) of studies. One study
employed a virus-induced RMS model. Overall, 40.0% (n = 30) of the studies
utilizing CDX models established alveolar RMS (aRMS), while 38.7% (n = 29) were
embryonal phenotypes (eRMS). There were 20.0% (n = 15) of studies that involved
a combination of both aRMS and eRMS subtypes. In one study (1.3%), the RMS
phenotype was spindle cell/sclerosing. Subcutaneous xenografts (n = 66, 55.9%)
were more frequently used compared to orthotopic models (n = 29, 24.6%).
Notably, none of the employed cell lines were derived from primary untreated
tumors. Only a minority of studies investigated disseminated RMS phenotypes
(n = 16, 13.6%). The utilization areas of RMS models included testing drugs (n =
64, 54.2%), studying tumorigenesis (n = 56, 47.5%), tumor modeling (n = 19,
16.1%), imaging (n = 9, 7.6%), radiotherapy (n = 6, 5.1%), long-term effects related
to radiotherapy (n = 3, 2.5%), and investigating biomarkers (n = 1, 0.8%). Notably,
no preclinical studies focused on surgery.
Conclusions: This up-to-date review highlights the need for mouse models with
dissemination phenotypes and cell lines from primary untreated tumors.
Furthermore, efforts should be directed towards underexplored areas such as
surgery, radiotherapy, and biomarkers. |
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Beschreibung: | Gefördert durch den Open-Access-Publikationsfonds der UB Marburg. |
DOI: | 10.3389/fonc.2024.1333129 |