File:Childhood adrenocortical tumours graph.jpg

Childhood Adrenocortical Tumours

Distribution of 125 cases of childhood adrenocortical tumour by age and gender.

"Childhood adrenocortical tumour (ACT) is not a common disease, but in southern Brazil the prevalence is 15 times higher than in other parts of the world. One hundred and thirty-seven patients have been identified and followed by our group over the past four decades. Affected children are predominantly girls, with a female-to-male ratio of 3.5:1 in patients below 4 years of age. Virilization alone (51.6%) or mixed with Cushing's syndrome (42.0%) was the predominant clinical picture observed in these patients. Tumours are unilateral, affecting both glands equally. TP53 R337H germline mutations underlie most childhood ACTs in southern Brazil. Epidemiological data from our casuistic studies revealed that this mutation has ~10% penetrance for ACT."

Original File Name: Figure 1. 1897-4287-4-2-81-1.jpg http://www.hccpjournal.com/content/4/2/81/figure/F1

Reference

<pubmed>20223012</pubmed>| Hered Cancer Clin Pract.

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