Adrenal medulla and paraganglia

McNicol, Anne Marie (2003). Adrenal medulla and paraganglia. In Ricardo V. Lloyd (Ed.), Endocrine Pathology. Differential Diagnosis and Molecular Advances 1 ed. (pp. 227-244) Totowa, N.J., USA: Humana Press. doi:10.1007/978-1-4419-1069-1_12


Author McNicol, Anne Marie
Title of chapter Adrenal medulla and paraganglia
Title of book Endocrine Pathology. Differential Diagnosis and Molecular Advances
Place of Publication Totowa, N.J., USA
Publisher Humana Press
Publication Year 2003
Sub-type Other
DOI 10.1007/978-1-4419-1069-1_12
Open Access Status Not yet assessed
Edition 1
ISBN 1588292088
Editor Ricardo V. Lloyd
Chapter number 12
Start page 227
End page 244
Total pages 18
Language eng
Subjects 11 Medical and Health Sciences
Abstract/Summary 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.
Keyword Paraganglioma
Sympathetic
Parasympathetic
Pheochromocytoma
Q-Index Code BX
Q-Index Status Provisional Code
Institutional Status Unknown
Additional Notes 2nd edition (2010) is published online via Springerlink

 
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Created: Wed, 17 Mar 2010, 00:52:30 EST by Laura McTaggart on behalf of Faculty Of Health Sciences