Paraganglioma

Tumours arising in clusters of neuroendocrine cells dispersed throughout the body

Aetiology

  • Can arise at any age but usually > 50
  • Can occur as part of genetic syndromes - MEN2, von Hippel-Lindau syndrome, neurofibromatosis type 1
    • Most common genetic cause of hereditary paragangliomas are mutations in the succinate dehydrogenase (SDH) subunit

Pathophysiology

  • Tumour are closely related to the autonomic nervous system, with either parasympathetic or sympathetic function
  • Sympathetic - generally arise in paraganglia below the level of the neck
    • Paravertebral - organ of Zuckerkandl, rarely bladder
  • Parasympathetic - related to great vessels of the head and neck
    • Carotid bodies, aortic bodies, jugulotypanic ganglia, ganglia nodosum of vagus and clusters around oral cavity, nose, nasopharynx, larynx and orbit

Histology

  • Nests of round/oval cells surrounded by delicate vascular septae
notion image

Clinical presentation

  • Sympathetic paragangliomas present with features of catecholamine excess e.g. such as headaches, palpitations, diaphoresis and hypertension
  • Parasympathetic paragangliomas present more commonly with mass effects such as cranial nerve palsies, a neck mass or tinnitus

Investigations

  • Imaging - CT, MRI, MIBG, PET

Management

  • Treatment may include surgical resection or radiotherapy