Paraganglioma

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Paraganglioma is a rare tumour.

General

  • Def'n: tumour of paraganglion (can be sympathetic or parasympathetic).
    • Most common paraganglioma - pheochromocytoma[1]
  • Head & neck most common site - after abdomen.
    • Carotid body tumour.

Epidemiology

  • Very rare
  • Rarely malignant
  • Familial syndromes assoc. with paragangliomas.[2]
    • von Hippel Lindau.
    • Hereditary paragangliomatosis.
    • Neurofibromatosis type 1 (von Recklinghausen disease).
    • MEN 2A.
    • MEN 2B.

Clinical

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

Microscopic

Features:[4]

  • Resembles pheochromocytoma
    • Zellballen (literally: "cell balls") - nests of cells.
    • Fibrovascular septae.
    • Finely granular cytoplasm (salt-and-pepper nuclei).

IHC

Features:[5]

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

See also

References

  1. EP P.327.
  2. EP. P.328.
  3. EP P.327.
  4. EP PP.329-332.
  5. EP P.335.