Adrenal cortical tumours: 25 years' experience at the Royal Children's Hospital, Melbourne

Aims: Adrenal cortical tumours remain a rare entity with inconsistent consensus about treatment and follow up. This article reviews 25 years of experience in the Royal Children's Hospital, Melbourne, Australia. Methods: All records with the diagnosis of adrenal adenoma or carcinoma between 1976 and 2001 were reviewed, excluding tumours of the adrenal medulla. Details were recorded for age, gender, family history, presentation, biochemistry, imaging, histology, diagnosis, treatment and outcome. Results: Twelve children (six boys, six girls) were diagnosed with adrenal cortical tumours in this period. Median age at diagnosis was 2.5 years (range 0.5-15.6 years). Six of the 12 children presented with virilization. The remaining six identified by ultrasound performed for hemi-hypertrophy (2), hypertension (2), and fever with abdominal pain (2). Five children had a family history of tumour and two of these five had a p53 mutation demonstrated on molecular genetic analysis. Tumours in five of the 12 children were defined as malignant on histology. Surgery was deemed curative in nine cases. Only one case required further surgery and two required chemotherapy. Time since diagnosis ranged from 1 to 25.8 years. Two children died from complications of the tumour. One other child died following development of a second tumour. Conclusion: Adrenal cortical tumours should always be considered in the differential for adrenal hormone excess. New information provided by mutational analysis may predict ongoing risks. Lifelong regular follow up is required.