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Hereditary pheochromocytoma-paraganglioma

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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

ICD-11: 5A75

OMIM: 115310 168000 171300 601650 618475 618464 605373 614165

UMLS: C4274332

GARD: 11984

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands Ελληνικά
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
English (2011) - PDQ Cancer Inf Sum
Guidelines
Emergency guidelines
Français (2023.pdf) - Orphanet Urgences
Español (2024.pdf) - Orphanet Urgences
Anesthesia guidelines
English (2016) - Orphananesthesia
Español (2016) - Orphananesthesia
Čeština (2016) - Orphananesthesia
Clinical practice guidelines
Français (2023) - Bull Cancer
English (2024) - Nat Rev Endocrinol
English (2016) - Eur J Endocrinol Logo ERN
English (2020) - Ann Oncol Logo ERN
English (2014) - J Clin Endocrinol Metab Logo ERN
Disease review articles
Review article
English (2006) - Orphanet J Rare Dis
Clinical genetics review
English (2023) - GeneReviews
Genetic testing
Guidance for genetic testing
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