Nine cases of childhood adrenal tumour presenting with hypertension and a review of the literature.
OBJECTIVE
To describe the clinical features, treatment and outcome of children adrenal tumors presenting with hypertension.


METHODS
The records of nine children under 16 years of age with adrenal tumours presenting with hypertension were analysed. Details were recorded for family history, clinical presentation, biochemistry, imaging, histology, treatment and outcome.


RESULTS
Abdominal mass was palpable only in one patient at diagnosis. Besides hypertension-associated symptoms, Cushing's syndrome was the common presentation form (n = 4). Abdominal computed topography showed adrenal mass in all patients. Tumours were completely resected for each patient. The median tumour weight was 73 g (11-530 g) and the size ranged from 1.5 x 1.5 to 12 x 14 cm2. Pheochromocytoma (n = 2), adrenocortical adenoma (n = 3), adrenocortical carcinoma (n = 1), neuroblastoma (n = 2) and ganglioneuromas (n = 1) were found. In one case, adrenal pheochromocytoma first occurred and non-functioning islet cell tumour successively occurred at pancreas. A better status is common at a median follow-up time of 3.5 years.


CONCLUSIONS
Childhood adrenal tumours presented with hypertension showed an atypical course, variable presentation. We report a unique case of adrenal pheochromocytoma followed by the occurrence of non-functioning islet cell tumour. Reversal of hypertension by surgery is crucial. Imaging techniques are important to detect adrenal tumours.
Wang
Xiumin
X
Department of Endocrinology, The Children's Hospital of Zhejiang, University School of Medicine, Hangzhou 310003, China. wangxiumin1019@126.com
Liang
Li
L
Jiang
Youjun
Y
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D000324:Adrenocorticotropic Hormone / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D006973:Hypertension / Q000209:etiology*; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D010673:Pheochromocytoma / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D012189:Retrospective Studies; D016896:Treatment Outcome; D014642:Vanilmandelic Acid / Q000097:blood
D014642:Vanilmandelic Acid; D000324:Adrenocorticotropic Hormone