• Ann. N. Y. Acad. Sci. · Aug 2006

    Review

    Paragangliomas: clinical overview.

    • William F Young.
    • Division of Endocrinology, Diabetes, Metabolism, Nutrition, and Internal Medicine, Mayo Clinic, 200 First Street S.W. Rochester, MN 55905, USA. Young.William@Mayo.edu
    • Ann. N. Y. Acad. Sci. 2006 Aug 1; 1073: 21-9.

    AbstractParagangliomas are rare tumors that arise from extra-adrenal paraganglia. The effective diagnosis and management of the paraganglioma patient involves the close collaboration of endocrinologists, endocrine surgeons, anesthesiologists, geneticists, laboratory specialists, radiologists, oncologists, and pathologists. Paragangliomas are diagnosed in the following clinical settings: signs and symptoms related to catecholamine hypersecretion, mass effect symptoms (e.g., with head and neck paragangliomas), incidental finding on imaging, or family screening for hereditary paraganglioma. Paragangliomas that hypersecrete catecholamines may cause signs and symptoms identical to those in patients with hyperfunctioning adrenal pheochromocytoma. When a catecholamine-secreting tumor is suspected in a patient because of paroxysmal symptoms, biochemical documentation of catecholamine and fractionated metanephrine hypersecretion should precede any form of imaging study. Catecholamine-secreting paragangliomas are found where chromaffin tissue is located (e.g., along the para-aortic sympathetic chain, or within the organs of Zuckerkandl at the origin of the inferior mesenteric artery, the wall of the urinary bladder, and the sympathetic chain in the neck or mediastinum). As many as 50% of paragangliomas are hereditary and may be associated with familial paraganglioma, neurofibromatosis Type 1, von Hippel-Lindau disease, the Carney triad, and, rarely, with multiple endocrine neoplasia Type 2. Genetic testing should be considered in all patients with paraganglioma. The treatment of choice for paraganglioma is surgical resection; most tumors are benign and can be excised totally. Following surgical cure, annual biochemical testing assesses for metastatic disease, tumor recurrence or delayed appearance of multiple primary tumors.

      Pubmed     Full text   Copy Citation     Plaintext  

      Add institutional full text...

    Notes

     
    Knowledge, pearl, summary or comment to share?
    300 characters remaining
    help        
    You can also include formatting, links, images and footnotes in your notes
    • Simple formatting can be added to notes, such as *italics*, _underline_ or **bold**.
    • Superscript can be denoted by <sup>text</sup> and subscript <sub>text</sub>.
    • Numbered or bulleted lists can be created using either numbered lines 1. 2. 3., hyphens - or asterisks *.
    • Links can be included with: [my link to pubmed](http://pubmed.com)
    • Images can be included with: ![alt text](https://bestmedicaljournal.com/study_graph.jpg "Image Title Text")
    • For footnotes use [^1](This is a footnote.) inline.
    • Or use an inline reference [^1] to refer to a longer footnote elseweher in the document [^1]: This is a long footnote..

    hide…