Hereditary pheochromocytoma-paraganglioma

Orpha code: 29072OMIM code: 614165

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.

Disease data
Classification

Disease

Synonyms
Familial pheochromocytoma-paraganglioma
Rodzinny guz chromochłonny - przyzwojak
ORPHA code
29072
OMIM code
614165
ICD10 code
D35.6
ICD11 code
-

No additional description.

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