Functioning adrenal tumours in children and adolescents: an institutional experience.
BACKGROUND
The purpose of the present paper was to carry out an audit of clinicopathological profile and treatment outcome in 13 children with functioning adrenal tumours.


METHODS
The medical records of 13 children with functioning adrenal tumours who were managed between June 1990 and January 1999 were reviewed. Demographic data, clinical features, biochemical and localization studies, operative details and follow-up records were studied. Children with neuroblastoma were excluded.


RESULTS
The mean age was 7.4 +/- 5.3 years. Seven patients had Cushing's syndrome (CS), two patients had virilizing tumours, three patients had phaeochromocytoma (PCC) and one patient had Conn's syndrome. All patients (except one child with CS) were treated surgically. Two children with adrenocortical carcinoma (ACCa) died during the perioperative period. Histopathological diagnosis was adrenal cortical adenoma (ACAd) in four patients, ACCa in five patients and PCC in three patients. Two ACCa patients died of metastases at 12 and 14 months, respectively, while the third is alive and well at 30 months. Children with ACAd are alive and well at 91, 56, 32 and 27 months postoperatively. Children with PCC are free of disease (normal urinary metanephrines) at 63, 18 and 8 months after surgery but require antihypertensive drugs in low doses.


CONCLUSIONS
The outcome of surgery is good in cases of ACAd and PCC. Although outcome is poor in ACCa, surgery remains the mainstay of treatment and offers good palliation.
Mishra
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Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226 014, India. skmishra@sgpgi.ac.in
Agarwal
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Misra
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Agarwal
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Mishra
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D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D000311:Adrenal Glands / Q000473:pathology; D000315:Adrenalectomy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009384:Paraneoplastic Endocrine Syndromes / Q000175:diagnosis / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D010673:Pheochromocytoma / Q000175:diagnosis / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D015996:Survival Rate