Androgen secreting adrenocortical tumours

Background-Androgen secreting adrenocortical tumours are rare in children a nd 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 andro gens was detected by assessment of serum androgen concentrations alone in f our 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 m easuring 5-9 cm, three were malignant and seven were benign, whereas all fo ur tumours > 10 cm were malignant. Histological slides were available for r eassessment in 25 children. Although mitoses and necrosis were more charact eristic of tumours with malignant behaviour, no exclusive histological feat ures of malignancy were seen. Conclusion-Histological criteria for malignancy are not reliable, whereas t umour size is important in assessing malignant potential.