Hereditary pheochromocytoma-paraganglioma

What is Hereditary pheochromocytoma-paraganglioma?

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.

Other condition names

  • Familial pheochromocytoma-paraganglioma

Inheritance type

Autosomal dominant

Age of Onset

  • Childhood
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