Urinary steroid profiling in the evaluation of patients with adrenocortical neoplasms.

Abstract

Background: Adrenocortical tumours are relatively common in childhood. Clinical presentation with an abdominal mass often signifies metastatic disease with poor prognosis. On the other hand, modern imaging techniques have significantly increased the pick up rate of adrenal masses (dubbed ‘incidentalomas’). Endocrine hypersecretion may result in varying degrees of vague symptomatology that may be clinically challenging to adequately evaluate. Objectives: To determine the urinary steroid profiles (by capillary gas chromatography) in a series of patients presenting with adrenocortical masses). Methods: The fifty patients included in this study had an adrenocortical tumour diagnosed on the basis of abdominal ultrasound, CT or at laparatomy. An aliquot of a 24 hours urine collection was analysed by capillary column gas chromatography after enzymatic hydrolysis and derivatization of constituent steroid metabolites. Twenty steroid derivative compounds were identified and quantified. Results: All 23 children (less than 16 years) in this series presented with endocrine symptoms with only 3 additionally presenting with a clinically evident abdominal mass. 11 out of 20 adult patients presented with Cushing’s syndrome, including 5 presenting with a clinically evident abdominal mass. Only one of the 50 patients studied here had a normal urinary steroid profile. The pattern or the amount of overall steroid metabolites excreted was abnormal in all the other patients. There was heterogeneity of urinary steroid profiles in both children (=<16 years) and adults. There were no profiles clearly distinguishing carcinoma from adenoma. In particular, 11-deoxy cortisol and its metabolites were not sufficiently discriminatory
to distinguish malignant from benign adrenocortical neoplasms: 16 of the 22 patients with carcinoma and 8 of the 15 patients with adenoma had raised excretion of 11- deoxycortisol metabolites. Conclusion: The pattern and quantities of urinary steroid metabolites are often a significantly deranged in both benign and malignant adrenocortical neoplasms. Such profiling should form part of the routine evaluation of adrenal masses (including ‘incidentalomas’) and Cushing’s syndrome in adults and precocity and virilism in childhood. The search for specific steroid metabolite patterns exclusively associated with malignant adrenocortical tumours remains elusive.