biostudies-literatureVerginelli FAssociazione Italiana per la Ricerca sul CancroMinistero dell’Istruzione, dell’Università e della RicercaNCI NIH HHS779-798https://www.ebi.ac.uk/biostudies/studies/S-EPMC5904229biostudies-literatureUnknown135(5)Tumours can be viewed as aberrant tissues or organs sustained by tumorigenic stem-like cells that engage into dysregulated histo/organogenetic processes. Paragangliomas, prototypical organoid tumours constituted by dysmorphic variants of the vascular and neural tissues found in normal paraganglia, provide a model to test this hypothesis. To understand the origin of paragangliomas, we built a biobank comprising 77 cases, 18 primary cultures, 4 derived cell lines, 80 patient-derived xenografts and 11 cell-derived xenografts. We comparatively investigated these unique complementary materials using morphofunctional, ultrastructural and flow cytometric assays accompanied by microRNA studies. We found that paragangliomas contain stem-like cells with hybrid mesenchymal/vasculoneural phenotype, stActa neuropathologicaParagangliomas arise through an autonomous vasculo-angio-neurogenic program inhibited by imatinib.PMC59042292015B7M39TP30 CA008748IG9168IG16932Guarnieri SMarchisio MOpocher GVeronese ASoderberg-Naucler CSanna MSheu ARajasekhar VKTramontana LVespa SRossi CVerginelli FBologna GPaties CTMorgano ACama ASchiavi FEsposito DLLanuti PD'Argenio APantalone MRFlorio RLotti LVTaschin EVisone RMariani-Costantini RPerconti SPrasad SCfalseParagangliomas arise through an autonomous vasculo-angio-neurogenic program inhibited by imatinib.Tumours can be viewed as aberrant tissues or organs sustained by tumorigenic stem-like cells that engage into dysregulated histo/organogenetic processes. Paragangliomas, prototypical organoid tumours constituted by dysmorphic variants of the vascular and neural tissues found in normal paraganglia, provide a model to test this hypothesis. To understand the origin of paragangliomas, we built a biobank comprising 77 cases, 18 primary cultures, 4 derived cell lines, 80 patient-derived xenografts and 11 cell-derived xenografts. We comparatively investigated these unique complementary materials using morphofunctional, ultrastructural and flow cytometric assays accompanied by microRNA studies. We found that paragangliomas contain stem-like cells with hybrid mesenchymal/vasculoneural phenotype, st2018-01-01T00:00:00Z2018 May2025-04-04T10:09:57.214Z2019-03-26T23:31:41ZS-EPMC59042292930572110.1007/s00401-017-1799-2