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. 2020 Sep 14;140(6):893–906. doi: 10.1007/s00401-020-02218-7

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© The Author(s) 2020

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

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Fig. 1 — Developmental, clinical and radiological characteristics of paragangliomas. a Paraganglia develop from neural crest cell precursor cells that migrate to various body sites. Different subpopulations are recognized that differ in their spatial predominance and capability of giving rise to functional or non-functional paragangliomas. b Distribution and frequency of paragangliomas in the human body: cauda equina paragangliomas are exceptionally rare. c Common sites of paraganglioma origin in detail with type of precursor neural crest cell population indicated by color. d Typical MRI presentation of a paraganglioma in the cauda equina region as a circumscribed, oval-shaped, contrast-enhancing mass at L2 on a sagittal T1 image with contrast agent. e Paraganglioma with unusual dissemination up to the thoracic spine upon tumor recurrence (sagittal T1 image with contrast agent). More often, dissemination occurs within cauda equina nerve roots in the lumbar compartment. f Same patients as in (e) with a paraganglioma metastasis in the cerebellopontine angle—mimicking a glomus jugulare tumor (axial T1 image with contrast agent)