APA
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Kemkem, Y., Quinn, M., Kövér, B., Santambrogio, A., Kaufman-Cook, J., Sherwin, O., … Andoniadou, C. (2025). Phaeochromocytomas and paragangliomas harbour tumour-initiating SOX2+ stem cells. BioRxiv.
Chicago/Turabian
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Kemkem, Y., Mark Quinn, B. Kövér, A. Santambrogio, J. Kaufman-Cook, O. Sherwin, L. Scriba, et al. “Phaeochromocytomas and Paragangliomas Harbour Tumour-Initiating SOX2+ Stem Cells.” bioRxiv (2025).
MLA
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Kemkem, Y., et al. “Phaeochromocytomas and Paragangliomas Harbour Tumour-Initiating SOX2+ Stem Cells.” BioRxiv, 2025.
BibTeX Click to copy
@article{y2025a,
title = {Phaeochromocytomas and paragangliomas harbour tumour-initiating SOX2+ stem cells},
year = {2025},
journal = {bioRxiv},
author = {Kemkem, Y. and Quinn, Mark and Kövér, B. and Santambrogio, A. and Kaufman-Cook, J. and Sherwin, O. and Scriba, L. and Brempou, D. and Segoviano, Miriam Vazquez and Cameron, Dylan and Berger, I. and Ng, Wen and Nonaka, Daisuke F and Theodoropoulou, M. and Pamporaki, C. and Carroll, Paul V. and Izatt, L. and Chapple, Paul J. and Bornstein, S. and Bechmann, N. and Steenblock, C. and Oakey, Rebecca J. and Andoniadou, C.}
}
Phaeochromocytomas (PCCs) and paragangliomas (PGLs), are rare neuroendocrine tumours that arise in the neural crest (NC)-derived adrenal medulla and the paraganglia, respectively. Approximately 10%-15% of patients with PCCs and 35%-40% with PGLs go on to develop metastatic disease, leading to a reported median overall survival of 7 years. The development of prognostic markers and subsequent personal therapeutic strategies are hindered by a lack of understanding of tumourigenesis. In other organs, cells with stem-like properties are at the root of tumour initiation and maintenance, due to their ability to self- renew and give rise to differentiated cells. We have recently shown that, in the human adrenal, a subset of sustentacular cells, endowed with a support role, are in fact SOX2+ postnatal adrenomedullary stem cells, that are specified along the neural crest migratory route. In this study, we intended to determine if SOX2+ cells in PCCs and PGLs can behave as tumour-initiating stem cells. Using expression and transcriptomic studies, we demonstrate the presence of SOX2/SOX2-expressing cells across a broad range of PCCs and PGLs, irrespective of tumour aggressiveness, location, and causative mutation. In silico analyses reveal the co-expression of SOX2 and chromaffin cell markers in the tumour, and the active proliferation of these double-positive cells. Isolation of these cells in vitro in stem cell-promoting media, and their xenotransplantation on chicken chorioallantoic membranes, demonstrates that they have the potential to expand and metastasise in ovo, supporting their potential as tumour-initiating cells.