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Adrenal Medulla and Paraganglia

  • Anne Marie McNicol
Chapter

Abstract

Paraganglia are of two types, associated with either the sympathetic or parasympathetic nervous systems. Tumors arising from these are paragangliomas. The term “pheochromocytoma” is reserved for intra-adrenal tumors and the others should be defined with respect to type and site. These are rare tumors, but it is now realized that more than 30% arise in the context of familial disease. In addition to the well-recognized associations with multiple endocrine neoplasia types 2A and 2B, von Hippel–Lindau syndrome, and neurofibromatosis type 1, the familial paraganglioma syndromes related to mutations in the genes encoding the B, C, and D subunits of succinate dehydrogenase have now been defined.

The diagnosis of malignancy is reserved for the presence of metastases in sites where paraganglial tissue would not be found. Local invasive growth may be lethal, but does not correlate with metastatic behavior. The identification of malignant potential in primary tumors can be difficult. Two recent multifactorial approaches show some promise in identifying tumors with a higher risk of metastasis, but benign behavior cannot be predicted with certainty. Emerging data from molecular genetic studies may be of help in future.

Keywords

Paraganglioma Sympathetic Parasympathetic Pheochromocytoma 

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© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Molecular and Cellular Pathology, UQ Centre for Clinical ResearchUniversity of Queensland, The Royal Brisbane and Women’s HospitalHerstonAustralia

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