Affiliation:
1. Institute for Diabetes and Cancer, Helmholtz Center Munich –
German Research Center for Environmental Health, Neuherberg,
Germany
2. Department of Biology and Biotechnology, University of Pavia, Pavia,
Italy
Abstract
AbstractResearch 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.