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Sporadic pheochromocytoma/secreting paraganglioma

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Disease definition

A rare, isolated, non-familial pheochromocytoma/paraganglioma tumor arising from neuroendocrine chromaffin cells of the adrenal medulla (pheochromocytoma) or from extra-adrenal chromaffin tissue (paraganglioma). The majority of these tumors are benign and the presenting symptoms are typically caused by the increased catecholamine production of the tumor, including hypertension (often paroxysmal), tachycardia, anxiety and/or excessive sweating.

ORPHA:276621

Classification level: Disorder

Prevalence: Unknown

Inheritance: Not applicable

Age of onset: Adolescent, Adult, Childhood

ICD-10: D35.0 , C74.1

ICD-11: 5A75

UMLS: C4707333

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
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
Genetic testing
Guidance for genetic testing
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.