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Open Access Green: Postprint online available 01/2025
as soon as is submitted to ZB.
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
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
ISSN (print) / ISBN
0018-5043
e-ISSN
1439-4286
Journal
Hormone and Metabolic Research
Quellenangaben
Volume: 56,
Issue: 1,
Pages: 51-64
Publisher
Thieme
Publishing Place
Rudigerstr 14, D-70469 Stuttgart, Germany
Non-patent literature
Publications
Reviewing status
Peer reviewed
Institute(s)
Institute of Diabetes and Cancer (IDC)
Grants
Deutsche Forschungsgemeinschaft (CRC/Transregio 205 The Adrenal: Central Relay in Health and Disease)