Paraganglioma

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Paraganglioma is a rare tumour arising from the paraganglion. A paraganglioma arising in the adrenal gland is known as a pheochromocytoma.

General

  • Definition: tumour of paraganglion.
    • Can be sympathetic or parasympathetic.
  • Most common paraganglioma = pheochromocytoma.[1]
    • Head & neck most common site - after abdomen.
  • Carotid body tumour = paraganglioma of carotid body.

Epidemiology

Clinical

  • 10% bilateral, multiple, familial, pediatric and malignant.[3]

Microscopic

Features:[4]

  • Zellballen (literally: "cell balls") - nests of cells.
  • Fibrovascular septae.
  • Finely granular cytoplasm (salt-and-pepper nuclei).
  • +/-Hemorrhage - very common.

DDx:

Images:

IHC

Features:[5]

  • Chromogranin +ve.
  • Synaptophysin +ve.
  • S100 +/-.
  • Cytokeratin -ve.
  • EMA -ve.
    • +ve in RCC.

EM

Features:[6]

  • Neurosecretory granules.
    • Electron dense core.
    • Typically perinuclear location.

Image:

See also

References

  1. Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 327. ISBN 978-0443066856.
  2. Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 328. ISBN 978-0443066856.
  3. Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 327. ISBN 978-0443066856.
  4. Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 329-332. ISBN 978-0443066856.
  5. Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 335. ISBN 978-0443066856.
  6. 6.0 6.1 URL: http://path.upmc.edu/cases/case408.html. Accessed on: 16 January 2012.