Paragangliomas: Clinical overview

Research output: Chapter in Book/Report/Conference proceedingConference contribution

142 Citations (Scopus)

Abstract

Paragangliomas are rare tumors that arise from extraadrenal 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.

Original languageEnglish (US)
Title of host publicationAnnals of the New York Academy of Sciences
Pages21-29
Number of pages9
Volume1073
DOIs
StatePublished - Aug 2006

Publication series

NameAnnals of the New York Academy of Sciences
Volume1073
ISSN (Print)00778923
ISSN (Electronic)17496632

Fingerprint

Paraganglioma
Catecholamines
Tumors
Metanephrine
Imaging techniques
Testing
Neoplasms
Signs and Symptoms
Screening
Para-Aortic Bodies
Neck
Tissue
Inferior Mesenteric Artery
Multiple Endocrine Neoplasia Type 2a
von Hippel-Lindau Disease
Neurofibromatosis 1
Incidental Findings
Mediastinum
Genetic Testing
Pheochromocytoma

Keywords

  • Catecholamines
  • Metanephrines
  • Paraganglioma

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Young, W. F. (2006). Paragangliomas: Clinical overview. In Annals of the New York Academy of Sciences (Vol. 1073, pp. 21-29). (Annals of the New York Academy of Sciences; Vol. 1073). https://doi.org/10.1196/annals.1353.002

Paragangliomas : Clinical overview. / Young, William Francis.

Annals of the New York Academy of Sciences. Vol. 1073 2006. p. 21-29 (Annals of the New York Academy of Sciences; Vol. 1073).

Research output: Chapter in Book/Report/Conference proceedingConference contribution

Young, WF 2006, Paragangliomas: Clinical overview. in Annals of the New York Academy of Sciences. vol. 1073, Annals of the New York Academy of Sciences, vol. 1073, pp. 21-29. https://doi.org/10.1196/annals.1353.002
Young WF. Paragangliomas: Clinical overview. In Annals of the New York Academy of Sciences. Vol. 1073. 2006. p. 21-29. (Annals of the New York Academy of Sciences). https://doi.org/10.1196/annals.1353.002
Young, William Francis. / Paragangliomas : Clinical overview. Annals of the New York Academy of Sciences. Vol. 1073 2006. pp. 21-29 (Annals of the New York Academy of Sciences).
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