Non-Mutational Key Features in the Biology of Thymomas.

Simple Summary: Organotypic features such as intratumoral thymopoiesis make thymomas (THs) unique tumors. Their remarkable histological heterogeneity and low mutational burden have largely precluded personalized therapies. Although most thymomas do not harbor known oncogenic driver mutations, common...

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Publicado en:Cancers Vol. 16; no. 5; pp. 942 - 958
Autores principales: Küffer, Stefan, Müller, Denise, Marx, Alexander, Ströbel, Philipp
Formato: pictorial review tables/charts Journal Article
Publicado: MDPI Mar2024
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Mar2024
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      pub: MDPI
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        10.3390/cancers16050942
        175991766
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        atl: Non-Mutational Key Features in the Biology of Thymomas.
      aug:
        au:
          Küffer, Stefan
          Müller, Denise
          Marx, Alexander
          Ströbel, Philipp
        affil: Institute of Pathology, University Medical Center Göttingen, University of Göttingen, 37075 Göttingen, Germany
      sug:
        subj:
          Thymoma Therapy
          Mutation
          Molecular Biology
          Chromosome Aberrations
          RNA
          Methylation
          Gene Expression
          Apoptosis
          Proteomics
          Metabolomics
      ab: Simple Summary: Organotypic features such as intratumoral thymopoiesis make thymomas (THs) unique tumors. Their remarkable histological heterogeneity and low mutational burden have largely precluded personalized therapies. Although most thymomas do not harbor known oncogenic driver mutations, common non-mutational key features such as chromosomal, epigenetic, and metabolic alterations are emerging that converge into a limited number of critical cellular pathways that may provide opportunities for therapeutic interventions. In this review, we have attempted to integrate the existing knowledge of TH biology into a broader picture and highlight opportunities for targeted treatment options in these non-mutated tumors. Thymomas (THs) are a unique group of heterogeneous tumors of the thymic epithelium. In particular, the subtypes B2 and B3 tend to be aggressive and metastatic. Radical tumor resection remains the only curative option for localized tumors, while more advanced THs require multimodal treatment. Deep sequencing analyses have failed to identify known oncogenic driver mutations in TH, with the notable exception of the GTF2I mutation, which occurs predominantly in type A and AB THs. However, there are multiple alternative non-mutational mechanisms (e.g., perturbed thymic developmental programs, metabolism, non-coding RNA networks) that control cellular behavior and tumorigenesis through the deregulation of critical molecular pathways. Here, we attempted to show how the results of studies investigating such alternative mechanisms could be integrated into a current model of TH biology. This model could be used to focus ongoing research and therapeutic strategies.
      pubtype: Academic Journal
      doctype:
        pictorial
        review
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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