Paraganglioma

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Paraganglioma
Diagnosis in short

Paraganglioma. H&E stain.
LM Zellballen (nests of cells), fibrovascular septae, salt-and-pepper nuclei, +/-hemorrhage (very common)
LM DDx neuroendocrine tumour, pheochromocytoma (paraganglioma of the adrenal gland), gangliocytic paraganglioma
IHC chromogranin +ve, synaptophysin +ve, CD56 +ve
Gross dusky colour
Site abdomen (adrenal gland paraganglioma = pheochromocytoma), head and neck (carotid body tumour)
Syndromes von Hippel Lindau, hereditary paragangliomatosis, neurofibromatosis type 1 (von Recklinghausen disease), MEN 2A, MEN 2B, Carney-Stratakis syndrome, Carney triad
Prevalence uncommon
Prognosis usually good, rarely malignant

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.
    • Locations of paraganglia
      • Paravertebral (retroperitoneal)
      • Near the large blood vessels of the head and neck and base of skull
      • Scattered in other tissues
  • Most common paraganglioma = pheochromocytoma.[1]
    • Sites relate to locations of paraganglia
        • Head & neck most common - neck, ear, carotid body, base of skull
        • Retroperitoneal/abdomen
        • Bladder

Special site names

  • Carotid body tumour = paraganglioma of carotid body - very vascular - right near a major artery. Don't stick a needle in it.
  • Glomus tympanicum tumor = paraganglioma of the middle ear - pulsitile tintinitis and conductive hearing loss.
  • Pheochromocytoma - basically a 'paraganglioma' in the adrenal medulla

Epidemiology

  • Rare.
  • Rarely malignant.

Familial syndromes associated with paragangliomas:[2]

Other associations - not proven to be genetic:

Clinical

  • 10% bilateral, multiple, familial, pediatric and malignant.[7]

Gross

  • Dusky colour.

Note:

Image:

Microscopic

Features:[8]

  • Zellballen - nests of cells - key low power feature.
    • Zellballen is "cell balls" in German.
  • Fibrovascular septae and sustentacular cells (structural support cell).
  • Finely granular cytoplasm (salt-and-pepper nuclei).
  • +/-Hemorrhage - very common.

DDx:

Images

Carotid body tumour:

  • Paraganglioma - intermed. mag. (WC)

  • Paraganglioma - high mag. (WC)

  • Neck - Paraganglioma - nice Zeballen (SKB)

  • Neck Paraganglioma - Carotid Body Tumor (SKB)

  • Neck - Paraganglioma - Carotid Body Tumor (SKB)

Duodenal paraganglioma - uncommon location:

  • Paraganglioma - low mag. (WC)

  • Paraganglioma - very high mag. (WC)

  • Paraganglioma - chromogranin A - intermed. mag. (WC)

  • Paraganglioma - S100 - very high mag. (WC)

Retroperitoneal paraganglioma

  • Retroperitoneum - Paraganglioma - Prominent vascular component (SKB)

  • Retroperitoneum - Paraganglioma (SKB)

  • Retroperitoneum - Paraganglioma - florid atypia (SKB)

  • Retroperitoneum - Paraganglioma - large nests (SKB)

Ear paraganglioma "Glomus Tympanicum"

  • Ear - Paraganglioma - Glomus Tympanicum Tumor (SKB)

  • Ear - Paraganglioma - Glomus Tympanicum Tumor (SKB)

Bladder

  • Bladder - Paraganglioma - Presented as micturation syncope (SKB)

Other:

  • Pheochromocytoma - high mag. (WC)

www:

IHC

Features:[9]

  • Chromogranin +ve.
  • Synaptophysin +ve.
  • S100 +ve/-ve (+ve in sustentacular cells, not tumor cells)
  • Cytokeratin -ve.
  • EMA -ve.
  • ATRX normal/loss.
    • Loss of staining a poor prognosticator in pheochromocytoma/paraganglioma.[10]

Testing for heritable tumours:

  • SDHB (SDHx alterations).
  • FH (loss in HLRCC).
  • 2SC (positive in HLRCC).
  • CAIX (+ve in VHL-related).
  • Alpha-inhibin (+ve in VHL-related and SHDx alterations).

EM

Features:[11]

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

Image:

Sign out

SOFT TISSUE, LEFT/RIGHT CAROTID BODY, EXCISION:
- PARAGANGLIOMA (SIZE IN CM).
- NEGATIVE RESECTION MARGIN.

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. ↑ Blay, JY.; Blomqvist, C.; Bonvalot, S.; Boukovinas, I.; Casali, PG.; De Alava, E.; Dei Tos, AP.; Dirksen, U. et al. (Oct 2012). "Gastrointestinal stromal tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.". Ann Oncol 23 Suppl 7: vii49-55. doi:10.1093/annonc/mds252. PMID 22997454. http://annonc.oxfordjournals.org/content/23/suppl_7/vii49.full.
  4. ↑ Lefebvre, M.; Foulkes, WD. (Feb 2014). "Pheochromocytoma and paraganglioma syndromes: genetics and management update.". Curr Oncol 21 (1): e8-e17. doi:10.3747/co.21.1579. PMID 24523625.
  5. ↑ Orrego JJ, Chorny JA (October 2024). "Hereditary leiomyomatosis and renal cell cancer (HLRCC), pheochromocytoma (PCC)/paraganglioma (PGL) and germline fumarate hydratase (FH) variants". Endocrinol Diabetes Metab Case Rep 2024 (4). doi:10.1530/EDM-24-0073. PMC 11737469. PMID 39705504. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737469/.
  6. ↑ Zavoshi S, Lu E, Boutros PC, Zhang L, Harari A, Hatchell KE, Nielsen SM, Esplin ED, Ouyang K, Nykamp K, Wilde B, Christofk H, Shuch B (June 2023). "Fumarate Hydratase Variants and Their Association With Paraganglioma/Pheochromocytoma". Urology 176: 106–114. doi:10.1016/j.urology.2022.11.053. PMID 36773955.
  7. ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 327. ISBN 978-0443066856.
  8. ↑ 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.
  9. ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 335. ISBN 978-0443066856.
  10. ↑ Wang LL, Wei XJ, Zhang QC, Li F, Chen GY (December 2025). "Analysis of clinicopathological and immunohistochemical features of pheochromocytoma/paraganglioma". Ann Diagn Pathol 79: 152525. doi:10.1016/j.anndiagpath.2025.152525. PMID 40652840.
  11. ↑ 11.0 11.1 URL: http://path.upmc.edu/cases/case408.html. Accessed on: 16 January 2012.
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