Paraganglioma

Paraganglioma is a rare tumour.

General

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

Epidemiology

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

Clinical

• 10% bilateral, multiple, familial, pediatric and malignant

Microscopic

Features:

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

IHC

Features:

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

See also

• Pheochromocytoma.