Heterogeneity of urinary steroid profiles in children with adrenocortical tumors.
The excretory patterns of urinary steroids determined by capillary gas chromatography in 11 children (aged 0.8-16.5 years) with adrenocortical tumors were established. In 8 patients the predominant clinical feature was virilization, in 3 others, Cushing's syndrome. In 5 patients (3 carcinoma, 2 adenoma) very high excretion of 3 beta-hydroxy-5-ene steroids was observed. In 2 others (adenomas) only moderately elevated excretion of 11 beta-hydroxyandrosterone was found. In 1 patient (adenoma) pregnanediol dominated in the steroid profile, accompanied by moderately elevated 3 beta-hydroxy-5-ene steroids. Out of 3 Cushingoid patients (1 carcinoma, 2 adenomas), 1 presented an atypical urinary steroid pattern for hypercortisolemia, without 5 alpha-reductase and 11 beta-hydroxysteroid dehydrogenase deficiencies. Neither the urinary steroid pattern nor tumor size alone were reliable indicators of tumor malignancy, as evaluated by a pathological examination and subsequent metastasis-free survival.
Małunowicz
E M
EM
Department of Laboratory Diagnostics, Child Health Center, Warsaw, Poland.
Ginalska-Malinowska
M
M
Romer
T E
TE
Ruszczyńska-Wolska
A
A
Dura
M
M
D000236:Adenoma / Q000652:urine; D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000652:urine*; D014408:Biomarkers, Tumor; D002277:Carcinoma / Q000652:urine; D002648:Child; D002675:Child, Preschool; D002849:Chromatography, Gas; D003480:Cushing Syndrome / Q000652:urine; D005260:Female; D006801:Humans; D007223:Infant; D013256:Steroids / Q000652:urine*; D013534:Survival
D014408:Biomarkers, Tumor; D013256:Steroids