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In the current review, we discuss findings related to the identification of normal chromaffin stem cells and CSCs, pathways involved in regulating the development of CSCs, and the importance of the stem cell niche in development and maintenance of CSCs in PCCs/PGLs. Additionally, we examine the development and feasibility of novel CSC-targeted therapeutic strategies aimed at eradicating especially recurrent and metastatic tumors. Copyright © 2020 Scriba, Bornstein, Santambrogio, Mueller, Huebner, Hauer, Schedl, Wielockx, Eisenhofer, And
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