Adrenocortical carcinoma is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Adrenocortical carcinoma, or a subtype of Adrenocortical carcinoma, affects less than 200,000 people in the US population.
Ophanet, who are a consortium of European partners, currently defines a condition rare when if affects 1 person per 2,000. They list Adrenocortical carcinoma as a "rare disease". More information about Adrenocortical carcinoma is available from Orphanet
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Adrenal hypofunction occurs when more than 90% of both adrenal glands are destroyed, an occurrence that typically results from an autoimmune process in which circulating antibodies react specifically against the adrenal tissue. Other causes include tuberculosis (once the chief cause; now responsible for less than 10% of adult cases), bilateral adrenalectomy, hemorrhage into the adrenal gland, neoplasms, and infections (acquired immunodeficiency syndrome, histoplasmosis, and cytomegalovirus). Rarely, a familial tendency to autoimmune disease predisposes the patient to adrenal hypofunction and other endocrinopathies.
Secondary adrenal hypofunction that results in glucocorticoid deficiency can stem from hypopituitarism (causing decreased corticotropin secretion), abrupt withdrawal of long-term corticosteroid therapy (long-term exogenous corticosteroid stimulation suppresses pituitary corticotropin secretion and results in adrenal gland atrophy), or removal of a nonendocrine, corticotropin-secreting tumor. Adrenal crisis follows when trauma, surgery, or other physiologic stress exhausts the body’s stores of glucocorticoids in a person with adrenal hypofunction.
Adrenal hypofunction affects 1 in 16,000 neonates congenitally. In adults, it affects 8 in 100,000 people, and males and females are affected equally. There’s no racial predilection.
Source: Professional Guide to Diseases (Eighth Edition), 2005
The term 'prevalence' of Adrenocortical carcinoma usually refers to the estimated population of people who are managing Adrenocortical carcinoma at any given time. The term 'incidence' of Adrenocortical carcinoma refers to the annual diagnosis rate, or the number of new cases of Adrenocortical carcinoma diagnosed each year. Hence, these two statistics types can differ: a short-lived disease like flu can have high annual incidence but low prevalence, but a life-long disease like diabetes has a low annual incidence but high prevalence. For more information see about prevalence and incidence statistics.
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