Adrenal gland and paraganglia

Author: Nat Pernick, M.D. (see Authors page)

Revised: 21 January 2016, last major update February 2005

Copyright: (c) 2002-2016,, Inc.

PubMed Search: Paraganglioma [title] adrenal

Related: Aorticopulmonary, Carotid body, Cauda equina, Jugulotympanic, Laryngeal, Pigmented
Cite this page: Paraganglioma - general. website. Accessed June 25th, 2017.
Definition / general
  • Tumors of paraganglia, regardless of location, although paraganglioma of adrenal medulla is termed pheochromocytoma
  • 5 - 10% occur outside adrenal gland
  • Associated with Carney syndrome, MEN syndromes, neurofibromatosis type 1, von Hippel-Lindau disease
  • 20% are multiple versus 10% for sporadic pheochromocytomas
  • 10 - 40% are malignant, may recur or metastasize to regional lymph nodes, bone, liver or lung; may cause death
  • May have cardiovascular symptoms due to toxic effect of norepinephrine on myocardium
  • Chemodectoma: carotid and aortic body tumor (tumors of paraganglia with a chemoreceptor function)
  • Familial cases: autosomal dominant with paternal imprinting, linkage to 11q23 and 11q13 and usually carotid body paragangliomas
    • Also other sites
  • Sympathoadrenal: associated with MEN 2a and 2b
Prognostic factors
  • Only definitive criteria for malignancy is presence of tumor at site where no normal paraganglionic tissue exists
  • Possibly necrosis and vascular invasion
  • Site: Organ of Zuckerkandl (22% - 50% malignant vs. 10%+ in adrenal gland), high mitotic activity
Case reports
Gross description
  • Rubbery, firm, may have pseudocapsule, but adherent to adjacent vessels
  • Brown cut surface; variable central scar
Gross images

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Retroperitoneal tumor

Microscopic (histologic) description
  • Same as pheochromocytoma
  • Nesting (zellballen) or trabecular pattern of cells within a prominent vascular network
  • Zellballen pattern may be obscured by anastomosing bands
  • Nests composed of round / oval cells and giant multinucleated cells with abundant granular eosinophilic or basophilic cytoplasm, may have nuclear atypia and vascular invasion (does not indicate malignancy)
  • May have dysmorphic vessels, melanin-like pigment, abundant stroma and osseous metaplasia
  • Intracytoplasmic hyaline globules are present in sympathoadrenal paragangliomas
  • No mitotic figures except in obviously malignant tumors; no rosettes or acini
  • Usually no chronic inflammatory infiltrate, no necrosis (unless preoperative tumor embolization)
Microscopic (histologic) images

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Various images

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Encapsulated mass with zellballen pattern

Cytology description
  • Irregular clusters of tumor cells with eosinophilic finely granular cytoplasm and mildly irregular nuclei
  • May have marked atypia
  • Fine needle aspiration not recommended for carotid body paragangliomas due to high likelihood of misinterpretation and risk for catecholamine crisis and hemorrhage
  • Same as pheochromocytoma
  • Reticulin highlights nesting pattern
  • Cauda equina tumors are usually keratin negative
Electron microscopy description
  • Chief cells have abundant cytoplasmic neurosecretory granules
  • May have giant mitochondria with paracrystalline inclusions
  • Sustentacular cells wrap around chief cells and lack neurosecretory granules
  • No desmosomes
Molecular / cytogenetics description
See also
Mediastinal paraganglioma
  • General:
    • Usually in anterosuperior mediastinum near aortic arch
    • Also in costovertebral sulcus
    • In one study, aggressive behavior with substantial morbidity or death in 45%

Organ of Zuckerkandl paraganglioma
  • General:
    • Close to normal location of this structure, near anterior wall of aorta and origin of inferior mesenteric artery
    • May invade inferior vena cava
  • Micro description
    • Often nuclear pseudoinclusions (invagination of cell cytoplasm)

Vagal paraganglioma
  • General:
    • Usually women in 30s and 40s
    • Near neck, jugular foramen with extension to base of skull
    • May cause deviation of palatine tonsil or vagus nerve palsy with hoarseness and dysphagia
  • Gross description:
    • Usually well-circumscribed, but may invade locally
    • Mean 4 cm, range 2 - 6 cm
  • Micro description:
    • Often prominent hyalinized fibrous septa