Epidemiology

Phaeochromocytoma is a rare condition; the annual incidence in the general population is less than 1 per 100,000 person-years.[11][12]​​​[13][14][15]​ An annual incidence of 500-1600 phaeochromocytoma or paraganglioma (PPGL) cases has been reported in the US.[16]

PPGL prevalence in the US is estimated to be between 1:2500 and 1:6500.[16] In the US Olmsted County population-study, the prevalence of phaeochromocytoma was 8 per 100,000 inhabitants (at 1/1/2017).[17]

Phaeochromocytoma is a rare cause of secondary hypertension, with a prevalence of <1% in a general hypertension outpatient clinic.[2][10]​ Autopsy studies show that 0.05% to 0.1% of patients have phaeochromocytomas, indicating that many cases may go undiagnosed or patients may be asymptomatic.[10] Up to 14% of patients with incidentally discovered adrenal masses on anatomical imaging prove to have a phaeochromocytoma.[10]

The prevalence is equal in men and women and is reported in people of all races. The peak incidence is in the third to fifth decades of life, with an average age at diagnosis of 43 years.[3] However, around 20% are identified in children, commonly associated with underlying genetic conditions.[3][18]

Up to one third of cases of phaeochromocytoma are associated with familial syndromes such as multiple endocrine neoplasia 2A and 2B, neurofibromatosis type 1, and Von Hippel-Lindau syndrome.[2][3][19]

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