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Steroid biochemistry of virilising adrenal tumours in childhood
Insights
Urinary steroid profiles in infants with virilizing adrenal tumors reveal diverse steroid biosynthesis patterns. Gas chromatography identified distinct steroid excretion patterns linked to tumor type and patient outcomes.
Area of Science:
- Endocrinology
- Pediatric Oncology
- Biochemistry
Background:
- Virilizing adrenal tumors in infants present a diagnostic challenge.
- Steroid excretion patterns can offer insights into tumor function.
Purpose of the Study:
- To analyze urinary steroid excretion in infants with virilizing adrenal tumors.
- To correlate steroid profiles with tumor histology and patient prognosis.
Main Methods:
- Gas chromatography was used to determine urinary steroid excretion in 12 infants.
- Patients were categorized into groups based on steroid excretion levels and profiles.
Main Results:
- Group A (high 17-oxosteroid excretion) showed dehydroepiandrosterone (DHA) dominance, with one case mimicking fetal steroid patterns.
- Group B (moderate 17-oxosteroid excretion) exhibited 11 beta-hydroxyandrosterone or pregnenolone metabolites.
- Tumor size and histology correlated with clinical outcomes; larger tumors were associated with poorer prognosis.
Conclusions:
- Urinary steroid profiles in virilizing adrenal tumors are heterogeneous.
- These distinct patterns reflect diverse in vivo steroid biosynthesis activities.
- Steroid analysis aids in understanding tumor behavior and predicting patient outcomes.
Abstract:
Steroid excretion in urine of 12 infants with virilising adrenal tumours has been determined using gas chromatography. In six children, (Group A, five female, one male) aged 2.8-5.3 years, very high urinary excretions of 17 oxosteroids (greater than 40 mumol/24 h) were largely accounted for by dehydroepiandrosterone (DHA). In one of the girls, the pattern of steroids excreted in urine was similar to that of newborn infants, with high excretions of 16-oxygenated derivatives of DHA. The histology of this tumour suggested a neoplasia of fetal-type adrenocortical cells. Very large tumours were found in three of the infants, two of whom have died and one has multiple metastases. From the other three children, small, well-encapsulated adenomas were successfully removed. Six children (Group B), had moderately elevated 17-oxosteroid excretions (8-17 mumol/24 h). In five of these cases (four female, one male) aged 0.8-5 years, 11 beta-hydroxyandrosterone was a consistently prominent urinary steroid. In one boy, aged 7.7 years, 17-oxosteroid excretion was 15 mumol/24 h and the major steroids in urine were metabolites of pregnenolone. These six children have survived with no clinical evidence of recurrent tumour. The in vivo functional activities of the tumours can be deduced from the different profiles of steroids in urine. These have revealed heterogeneous patterns of steroid biosynthesis.