Cushing's disease epidemiology [Neurosurgery Wiki]

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====== Cushing's disease epidemiology ======

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Cushing's disease, which is caused by an ACTH-secreting pituitary adenoma, is a rare endocrine disorder. Its epidemiology is characterized by the following points:

1. Incidence:
The incidence of Cushing's disease is estimated to be 1 to 2 cases per million people per year.
It accounts for approximately 70% of endogenous cases of ACTH-dependent Cushing's syndrome.
2. Prevalence:
The prevalence of Cushing's disease is estimated to be around 40 cases per million people.
3. Gender:
There is a female predominance, with women being affected approximately 3 to 8 times more frequently than men. This gender difference is most prominent in the reproductive years (ages 20-40).
Men tend to be diagnosed later in life, often with larger tumors.
4. Age of Onset:
Cushing's disease typically presents between the ages of 20 and 50, with the peak incidence occurring in the third and fourth decades of life.
It is less common in children but does occur, often linked to more aggressive tumor behavior in pediatric cases.
5. Geographic Distribution:
Cushing's disease has no specific geographic or ethnic predilection and can occur in individuals from all regions of the world.
6. Risk Factors:
There are no well-established environmental or lifestyle risk factors for developing Cushing’s disease, as it is largely a sporadic condition.
Some rare genetic conditions (e.g., multiple endocrine neoplasia type 1, Carney complex) may increase the risk of pituitary adenomas, including those causing Cushing’s disease.
Given its rarity, diagnosis of Cushing's disease can be delayed, often taking several years after the onset of symptoms. Awareness of the epidemiological characteristics helps guide clinicians in recognizing this rare but serious condition.
----


40 cases/million population. [[ACTH]]-producing adenomas comprise 10–12% of [[pituitary neuroendocrine tumor]]s
((Banasiak MJ, Malek AR. Nelson syndrome: compre- hensive review of pathophysiology, diagnosis, and management. Neurosurg Focus. 2007; 23)).
[[Cushing’s disease]] is 9 times more common in women, whereas ectopic ACTH production is 10 times more common in males. Non-iatrogenic CS is 25% as common as acromegaly.
At the time of presentation, over 50% of patients with Cushing’s disease have pituitary tumors < 5 mm in diameter, which are very difficult to image with CT or MRI. Most are basophilic, some (especially the larger ones) may be chromophobic. Only ≈ 10% are large enough to produce some mass effect, which may cause enlargement of the sella turcica, visual field deficit, cranial nerve involvement, and/or [[hypopituitarism]].
----

In 90% of the cases, [[pituitary neuroendocrine tumor]] is the etiological cause in [[Cushing's disease]]. 

[[Pituitary corticotroph adenoma]]s, comprising functional and [[silent corticotroph adenoma]]s, represent approximately 10%–15% of all [[pituitary neuroendocrine tumor]]s.

The incidence of [[Cushing's disease]] is reported to vary between 2.4 per million persons in Europe and 8 per million in those <65 years of age in the United States
((Broder MS, Neary MP, Chang E, et al. Incidence of Cushing's syndrome and Cushing's disease in commercially-insured patients <65 years old in the United States. Pituitary 2015; 18:283–289.))
((Colao A, Boscaro M, Ferone D, et al. Managing Cushing's disease: the state of the art. Endocrine 2014; 47:9–20.)).

Cushing's disease occurs more often in women than men and more often occurs between the ages of 20 and 40.

The prevalence of Cushing's disease is estimated to be 39 per 1,000,000 people
((Feelders RA, Pulgar SJ, Kempel A, Pereira AM: The burden of Cushing's disease: clinical and health-related quality of life aspects. Eur J Endocrinol 167:311–326, 2012 )).
Approximately 12,000 people affected in the United States alone. This number, however, may be much higher, given that Cushing's disease is frequently misdiagnosed and the diagnosis is often delayed
((Nieman LK, Biller BMK, Findling JW, Newell-Price J, Savage MO, Stewart PM, et al.: The diagnosis of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 93:1526–1540, 2008)).

===== Literature =====
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