Heart tumor
Cardiac tumors - benign

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

Revised: 19 March 2018, last major update May 2007

Copyright: (c) 2007-2018, PathologyOutlines.com, Inc.

PubMed Search: Paraganglioma heart
Page views in 2018: 265
Page views in 2019 to date: 12
Cite this page: Pernick, N. Paraganglioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/hearttumorparaganglioma.html. Accessed January 23rd, 2019.
Definition / general
  • Also called extra adrenal pheochromocytoma (if secretes catecholamines) or chemodectoma (at carotid body)
  • Either functional (secretes catecholamine, associated with hypertension, headache, flushing) or nonfunctional
  • Very rare
  • Left atrium or near aortic root, where intrinsic cardiac ganglia (chromaffin cells) are located
Case reports
  • Excision or transplantation
  • 5 - 10% recur or metastasize after excision
Gross description
  • 5 - 7 cm, unencapsulated; on epicardium but may infiltrate adjacent cardiac tissue
Gross images

Images hosted on Pathout server, contributed by. Dr. Debra Zynger, Ohio State University (USA):
Missing Image

Periaortic paraganglioma

Microscopic (histologic) description
  • Unencapsulated, organoid appearance (zellballen/nests of cells) surrounded by sustentacular cells
  • No necrosis, no/minimal mitotic figures
Microscopic (histologic) images

Nested cells in organoid pattern; right image with chromogranin

Pigmented tumor

Paraganglia cells within right atrium (normal)

Positive stains
Negative stains
Electron microscopy description
  • Norepinephrine-type granules, few epinephrine-type granules
Additional references