Orphanet: Hereditary pheochromocytoma paraganglioma

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.


Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

Hereditary pheochromocytoma-paraganglioma

Disease definition

Hereditary paraganglioma-pheochromocytomas (PGL/PCC) are rare neuroendocrine tumors represented by paragangliomas (occurring in any paraganglia from the skull base to the pelvic floor) and pheochromocytomas (adrenal medullary paragangliomas; see this term).


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: -

Detailed information

Article for general public


The documents contained in this web site 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.