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Chap 26 - PARAGANGLIOMAS

Published online by Cambridge University Press:  01 March 2011

By
Markku Miettinen
Edited by
Markku Miettinen
Markku Miettinen
Affiliation:
Armed Forces Institute of Pathology, Washington DC
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Summary

Paragangliomas include pheochromocytomas and other paraganglionic tumors that are neural autonomic nervous system-related tumors showing neural differentiation. They are presumably derived from paraganglionic cells. Gastrointestinal autonomic nerve tumor (GANT) is now classified as a gastrointestinal stromal tumor (GIST) and is discussed in Chapter 17. Neuroblastoma, a primitive childhood tumor with sympathetic nerve differentiation, and ganglioneuroma, the most differentiated member of the same family, are included in Chapter 31.

OVERVIEW OF THE PARAGANGLIA

The paraganglia are collections of specialized neural cells that serve neurosecretory and neuroreceptive functions at various sites, such as oxygen sensing in the carotid bodies. The paraganglia include the adrenal medulla, and small paraganglia along the para-aortic sympathetic nervous chains around the aorta in the abdomen and thorax (sympathetic paraganglia), and associated with the parasympathetic nervous system, especially in the neck associated with carotid arteries (carotid body) and the vagus nerve (vagal paraganglia). Organ-based paraganglia include the adrenal medulla and small clusters of paraganglionic cells that occur in the urinary bladder and the mesenteries, and around the gallbladder, heart, and lungs, among others.

Normal paraganglia are composed of neural cells, the chief cells that are typically clustered as spherical collections (“cell balls,” originally described as Zellballen in German texts. These structures are sometimes seen in surgical specimens, especially from the abdomen (Fig. 26.1).

The paraganglioma chief cells are surrounded by Schwann cell–like elongated cells, the so-called sustentacular cells.

Type
Chapter
Information
Modern Soft Tissue Pathology
Tumors and Non-Neoplastic Conditions
 , pp. 755 - 776
Publisher: Cambridge University Press
Print publication year: 2010

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References

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  • PARAGANGLIOMAS
  • Edited by Markku Miettinen, Armed Forces Institute of Pathology, Washington DC
  • Book: Modern Soft Tissue Pathology
  • Online publication: 01 March 2011
  • Chapter DOI: https://doi.org/10.1017/CBO9780511781049.027
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  • PARAGANGLIOMAS
  • Edited by Markku Miettinen, Armed Forces Institute of Pathology, Washington DC
  • Book: Modern Soft Tissue Pathology
  • Online publication: 01 March 2011
  • Chapter DOI: https://doi.org/10.1017/CBO9780511781049.027
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  • PARAGANGLIOMAS
  • Edited by Markku Miettinen, Armed Forces Institute of Pathology, Washington DC
  • Book: Modern Soft Tissue Pathology
  • Online publication: 01 March 2011
  • Chapter DOI: https://doi.org/10.1017/CBO9780511781049.027
Available formats
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