11-beta-hydroxyandrosterone and Adrenal-Cortex-Neoplasms

Androgen secreting adrenocortical tumours are rare in children and the determination of their malignant potential can be difficult.. To assess the presentation, histology, and clinical behaviour of these tumours.. Two tertiary referral centres.. Retrospective analysis of children diagnosed with an androgen secreting adrenocortical tumour between 1976 and 1996.. Twenty three girls and seven boys aged 0-14 years.. 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 androgens was detected by assessment of serum androgen concentrations alone in four 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 measuring 5-9 cm, three were malignant and seven were benign, whereas all four tumours > 10 cm were malignant. Histological slides were available for reassessment in 25 children. Although mitoses and necrosis were more characteristic of tumours with malignant behaviour, no exclusive histological features of malignancy were seen.. Histological criteria for malignancy are not reliable, whereas tumour size is important in assessing malignant potential.

Topics: Adolescent; Adrenal Cortex Neoplasms; Androgens; Androsterone; Biomarkers; Child; Child, Preschool; Female; Follow-Up Studies; Growth Disorders; Humans; Hydrocortisone; Infant; Male; Necrosis; Puberty, Precocious; Retrospective Studies; Virilism

Gas-chromatographic analysis of the urinary steroids in an 18 year-old girl with primary amenorrhoea and hirsutism revealed markedly elevated excretion of androsterone, etiocholanolone, dehydroepiandrosterone, and androstendiole, both under basal conditions and after oral dexamethasone (0.5 mg q.i.d. for three days). Adrenal scintigraphy revealed the presence of a tumour of the right adrenal gland, which was subsequently removed by unilateral adrenalectomy. Histologically, the tumour showed marked anisocytosis, but since there was no evidence of capsular or angioinvasion or of mitotic activity, it was classified as an adrenocortical adenoma. Postoperatively the patient showed regression of the virilizing syndrome and normal menstrual bleeding. Urinary steroid excretion has remained normal for four years after adrenalectomy. Androgen-producing tumours must be considered as a possible cause of hirsutism in young females and should, thus, be excluded in each case. Analysis of urinary steroids by gas chromatography is a valuable tool in accomplishing this task.

Topics: Adenoma; Adolescent; Adrenal Cortex Neoplasms; Androgens; Androstenediols; Androsterone; Chromatography, Gas; Dehydroepiandrosterone; Etiocholanolone; Female; Humans; Paraneoplastic Endocrine Syndromes; Pregnanediol; Pregnanetriol