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Hereditary pheochromocytoma-paraganglioma

Disease definition

A rare, hereditary, pheochromocytoma/paraganglioma tumor arising from neuroendocrine chromaffin cells of the adrenal medulla (pheochromocytoma) or from any paraganglia from the skull base to the pelvic floor (paraganglioma). Clinical manifestations are often linked to excess catecholamines production causing sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, palpitations, pallor and apprehension or anxiety. Hereditary pheochromocytoma/paraganglioma tumors tend to present at younger ages, to be multi-focal, bilateral, and recurrent, or to have multiple synchronous neoplasms.

ORPHA:29072

Classification level: Disorder
  • Synonym(s):
    • Familial pheochromocytoma-paraganglioma
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood
  • ICD-10: C74.1  C75.5  D35.0  D35.6
  • OMIM: 115310  168000  171300  601650  605373  614165  618464  618475
  • UMLS: -
  • MeSH: -
  • GARD: 11984
  • MedDRA: -

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