Epidemiology of Adrenocortical Carcinoma



It is generally accepted that adrenocortical carcinoma (ACC) is a rare disease. However, valid data on the exact incidence and prevalence of ACC are lacking. Adrenal masses are among the most frequent tumors in humans. The vast majority of these tumors are nowadays found incidentally, and the prevalence of these adrenal incidentalomas is estimated as at least 3% in a population over the age of 50 years and increases to as much as 10% in the elderly [1–5]. However, it is well established that about 80% of these incidentally detected adrenal masses represent hormonally inactive adrenal adenomas and only a small minority are ACCs [2, 4, 6]. Data obtained from the National Cancer Institute Survey from the early 1970s estimated an incidence of 1–2 per million population per year, leading to 0.2% of cancer deaths in the United States [7]. A more recent analysis of the SEER database, including data from 12 US states, indicated an annual age-adjusted incidence of 0.72 per million [8, 9]. However, data from the German ACC Registry suggest that the incidence is >1 per million (Fassnacht & Allolio, unpublished data) and may be even higher.


Congenital Adrenal Hyperplasia Adrenal Mass Adrenocortical Carcinoma Adrenal Metastasis Referral Bias 


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© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  1. 1.Department of Internal Medicine I, Endocrine and Diabetes UnitUniversity Hospital of WürzburgWürzburgGermany

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