Functional Adrenocortical Tumour in Young
2010
Adrenocortical tumours (ACTs) in children are extremely rare, accounting only for 0.3–0.4% of all neoplasms in this age [1]. Adrenocortical tumours mostly secrete hormones, resulting in Cushing's syndrome, virilisation, hyperaldosteronism or feminization, while the non-functioning ones are unusual. Two syndromes have a clear association with adrenocortical tumour : Li Fraumeni syndrome is associated with mutations of the p53 gene, and Beckwith Wiedemann syndrome which has mutations in the 11p15 region [2]. Here we are reporting a rare case of functional adrenocortical tumour in a three year old boy.
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