Cushing syndrome (CS) is a rare disease in children associated with weight gain and stunting of their linear growth. In older children, pituitary adenomas are a more common cause of CS. The clinical presentation of CS varies in children such as truncal obesity, striae, facial plethora, hypertension, and PKOS-like (polycystic ovary syndrome) feature. Here in we report an adolescent presented with obesity, short stature and late puberty but without metabolic syndrome or hirsutism and diagnosed as unilateral adrenal adenoma. A 15 years-old female patient evaluated for stature. There was a history of weight gain and stunting of her linear growth especially last three years. When examined she had a facial plethora, abdominal obesity, non-specifically maculopapuler rash on the extremities. Her height standard deviation score (SDS) was −4.3 (136.6 cm) and relative body weight was 145% (47.3 kg). Her breasts were at Tanner stage I with lipomastia. Her bone age was 11 years-old. On laboratory examinations were revealed that FSH 0.17 mIU/ml, LH 0.05 mIU/ml with low estradiol levels. Plasma testosterone (101 ng/dl; N: 655) and 24-h urinary free cortisol level were increased (829 mcg/m2 per day; N<70) with suppressed adrenocorticotrophic hormone (<5 pg/ml) level. Plasma levels of androstenodione (0.76 ng/dl; N:<2.0), dehydroepiandrosterone sulfate (57.5 μg/dl; N: 17350), and 17-hydroxyprogesterone (0.5 ng/ml; N:<2) were normal. Abdominal imaging showed a right surrenal mass, which was 3.0 cm axis. Laparoscopic surgery was performed to resect the mass. Histologically, the neoplasm was diagnosed as an adrenocortical oxyphilic adenoma. There was a clinically and laboratory full recovery after surgery. After 6 months of surgery, her weight loss was 9 kg, and height gain was 4.4 cm. CS and adrenal adenoma should be kept in mind in patient with short stature with obese children and late puberty adolescent. All hormones secreted from the adrenal cortex should be measured in every patient with an adrenal tumor. Laparoscopic adrenalectomy is a safe and feasible, method for curative therapy for patient with surrenal adenoma.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology