Androgen secreting adrenocortical tumours

Abstract

Background: Androgen secreting adrenocortical tumours are rare in children and the determination of their malignant potential can be difficult.

Objectives: To assess the presentation, histology, and clinical behaviour of these tumours.

Setting: Two tertiary referral centres.

Study design: Retrospective analysis of children diagnosed with an androgen secreting adrenocortical tumour between 1976 and 1996.

Patients: Twenty three girls and seven boys aged 0-14 years.

Results: Pubic hair was observed in all children, clitoromegaly or growth of the phallus in 23 children, acceleration of linear growth in 22 children, and advanced bone age (> 1.5 years) in 18 children. Hypersecretion of androgens was detected by assessment of serum androgen concentrations alone in four patients and by 24 hour urine steroid excretion profiles in 22 patients. All 16 tumours measuring < 5 cm in diameter were benign. Of the tumours measuring 5-9 cm, three were malignant and seven were benign, whereas all four tumours > 10 cm were malignant. Histological slides were available for reassessment in 25 children. Although mitoses and necrosis were more characteristic of tumours with malignant behaviour, no exclusive histological features of malignancy were seen.

Conclusion: Histological criteria for malignancy are not reliable, whereas tumour size is important in assessing malignant potential.