Androgen secreting adrenocortical tumours
a The London Centre for Paediatric Endocrinology, The
Middlesex and Great Ormond Street Hospitals, London W1N 8AA, UK, b Department of
Chemical Pathology, University College London, London W1N 8AA, UK, c Department of Histopathology, St
Bartholomew's Hospital, London EC1A 7BE, UK, d Department of Paediatric Endocrinology, St
Bartholomew's Hospital
Correspondence to: Dr P C Hindmarsh, Cobbold Laboratories, The Middlesex Hospital, Mortimer Street, London W1N 8AA, UK.
Accepted 7 July 1998
BACKGROUND
Androgen secreting
adrenocortical tumours are rare in children and the determination
of their malignant potential can be difficult.
OBJECTIVESTo assess the presentation,
histology, and clinical behaviour of these tumours.
SETTINGTwo tertiary referral centres.
Study designRetrospective
analysis of children diagnosed with an androgen secreting
adrenocortical tumour between 1976 and 1996.
PATIENTSTwenty three girls and seven boys aged
0-14 years.
RESULTSPubic 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.
CONCLUSIONHistological criteria for malignancy
are not reliable, whereas tumour size is important in assessing
malignant potential.
© 1999 by Archives of Disease in Childhood
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