Adrenocortical tumors are rare childhood neoplasms. More than 95% are functional and present with virilization, Cushings syndrome, hypertension, or hyperestrogenism. We present a exceptionally rare case of a patient with androgen- and cortisol-co-secreting adrenal adenoma. A 4-year-old girl was referred to us for appearance of symptoms of virilization: moustache, pubic hair, and gradual enlargement of clitoris for 1 year. Her voice gardually deepened and changed to male pattern. On systemic examination, her weight was 26 kg, height was 112 cm, BMI 17.46. Pulse was 100 beats/min, blood pressure was 100/60 mmHg. She had no Cushingoid features. The laboratory showed that she had elavated 24 h urine free cortisol level: 362.7 μg/dl (21143 μg/dl) and serum testosteron: 286.5 ng/dl (1476 ng/dl). The serum ACTH was low: 1.58 pg/ml (7.263.3 pg/ml). Levels of DHEAS were normal: 0.914 μg/ml (0.53.5 μg/ml). Levels of 17OH(P), LH, FSH, estrogen were normal range. The bone-age was 11 years. Abdominal CT scan showed a left adrenal mass. It was decided to manage her with surgery to remove the tumor. Subsequently adrenalectomy was performed and histopathology study revealed a 5.5×5×3.5 adrenal adenoma. Follow-up of the patient showed that signs of virilization were suppressed. Serum testosterone levels dropped to normal after surgery, and remained normal. Two to 6 months after adrenalectomy, she was noticed to have significant symptoms of adrenal insufficiency and gradual enlargement of breast (Tanner B2). Laboratory tests showed: AM cortisol levels and ACTH levels were low on several occasions. The tests of diagnosis for precocious puberty (PP) were performed. After confirming the diagnosis adrenal insufficiency and PP the patient was given 5 mg of hydrocortisone a day and 3.75 mg of Diphereline a month. In summary, a diagnosis of adrenal cortical adenoma requires surgical removal as early as possible to prevent the untoward effects of virilization or corticosteroid excess. Although the girl in the present study seems to have been cured, long-term follow-up is warranted.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology