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.
Impact Factor
Scopus SNIP
Web of Science
Times Cited
Scopus
Cited By
Altmetric
Publication type
Article: Journal article
Document type
Review
Thesis type
Editors
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
Keywords plus
Language
english
Publication Year
2024
Prepublished in Year
0
HGF-reported in Year
2024
ISSN (print) / ISBN
0018-5043
e-ISSN
1439-4286
ISBN
Book Volume Title
Conference Title
Conference Date
Conference Location
Proceedings Title
Quellenangaben
Volume: 56,
Issue: 1,
Pages: 51-64
Article Number: ,
Supplement: ,
Series
Publisher
Thieme
Publishing Place
Rudigerstr 14, D-70469 Stuttgart, Germany
Day of Oral Examination
0000-00-00
Advisor
Referee
Examiner
Topic
University
University place
Faculty
Publication date
0000-00-00
Application date
0000-00-00
Patent owner
Further owners
Application country
Patent priority
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)
Copyright
Erfassungsdatum
2024-01-07