Adrenocortical Carcinoma in Childhood

Patients younger than 16 years with adrenal neoplasms are more likely to have malignant tumors than adults. A survey of the English literature between 1956 and 1986 provided 209 cases of children with adrenocortical neoplasms,39 66% of which were malignant. Average size and weight of malignant versus benign tumors were 9 versus 4 cm and 466 versus 43 g, respectively. The female/male ratio was 2.2:1, and the mean age at presentation was 4.6 years (range, 5 days to 16.5 years). Hirsutism was the most common presenting symptom (51%), followed by hypercortisolism (30%) and feminization (10%); 8% of the tumors were nonfunctional. Of interest is the association with congenital abnormalities such as hemihypertrophy, Beckwith-Wiedemann syndrome, vascular malformations, urologie abnormalities, and tumors of the central nervous system. Adrenal neoplasms have also been reported in patients with salt-losing congenital hyperplasia.39

The biochemical profile in children is similar to that in adults. Surgery is the only means offering cure. The role of adjuvant therapy is unproved. Average survival is 24 months but can reach up to 8 years. It should also be kept in mind that 40% of neuroblastomas are located in the adrenals and are now commonly diagnosed by antenatal ultrasonography.

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