Animal and cell culture models of PPGLs - Achievements and limitations.
    
    
        
    
    
        
        Horm. Metab. Res. 56, 51-64 (2024)
    
    
    
      
      
	
	    Research on rare tumors heavily relies on suitable models for basic and translational research. Paragangliomas (PPGL) are rare neuroendocrine tumors (NET), developing from adrenal (pheochromocytoma, PCC) or extra-adrenal (PGL) chromaffin cells, with an annual incidence of 2-8 cases per million. While most PPGL cases exhibit slow growth and are primarily treated with surgery, limited systemic treatment options are available for unresectable or metastatic tumors. Scarcity of appropriate models has hindered PPGL research, preventing the translation of omics knowledge into drug and therapy development. Human PPGL cell lines are not available, and few animal models accurately replicate the disease's genetic and phenotypic characteristics. This review provides an overview of laboratory models for PPGLs, spanning cellular, tissue, organ, and organism levels. We discuss their features, advantages, and potential contributions to diagnostics and therapeutics. Interestingly, it appears that in the PPGL field, disease models already successfully implemented in other cancers have not been fully explored.
	
	
	    
	
       
      
	
	    
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        Publication type
        Article: Journal article
    
 
    
        Document type
        Review
    
 
    
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        Keywords
        pheochromocytoma; paraganglioma; PDX; SDHx; neuroendocrine tumor; Metastatic Pheochromocytoma; Molecular Characterization; Paraganglioma Xenografts; Transcriptome Analysis; Signaling Pathways; Adrenal-gland; Mouse Model; Growth-rate; In-vivo; Sdhb
    
 
    
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        Language
        english
    
 
    
        Publication Year
        2024
    
 
    
        Prepublished in Year
        0
    
 
    
        HGF-reported in Year
        2024
    
 
    
    
        ISSN (print) / ISBN
        0018-5043
    
 
    
        e-ISSN
        1439-4286
    
 
    
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	    Volume: 56,  
	    Issue: 1,  
	    Pages: 51-64 
	    Article Number: ,  
	    Supplement: ,  
	
    
 
    
        
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            Publisher
            Thieme
        
 
        
            Publishing Place
            Rudigerstr 14, D-70469 Stuttgart, Germany
        
 
	
        
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        Reviewing status
        Peer reviewed
    
 
     
    
        POF-Topic(s)
        30201 - Metabolic Health
90000 - German Center for Diabetes Research
    
 
    
        Research field(s)
        Helmholtz Diabetes Center
    
 
    
        PSP Element(s)
        G-502590-001
G-501900-251
    
 
    
        Grants
        Deutsche Forschungsgemeinschaft (CRC/Transregio 205 The Adrenal: Central Relay in Health and Disease)
    
 
    
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        Erfassungsdatum
        2024-01-07