Paragangliomas: Clinical overview

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

145 Scopus citations

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 publicationPheochromocytoma
Subtitle of host publicationFirst International Symposium
PublisherBlackwell Publishing Inc.
Pages21-29
Number of pages9
ISBN (Print)1573315974, 9781573315975
DOIs
StatePublished - Aug 2006

Publication series

NameAnnals of the New York Academy of Sciences
Volume1073
ISSN (Print)0077-8923
ISSN (Electronic)1749-6632

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Keywords

  • Catecholamines
  • Metanephrines
  • Paraganglioma

ASJC Scopus subject areas

  • Neuroscience(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • History and Philosophy of Science

Cite this

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