Adrenal cortical tumor is rare in children and aldolescents. It is more common in girls and almost hormone overproduction with Cushing syndrome and virilization. Resection is the main management in order to control defenitively. Differentiating from begnin to malignancy lesion still remains a big obstacle for the clinicians and histopathologist. We report two cases of adrenal cortical tumor in children with different signs and symptoms and review their clinical presentations, pathology and follow-up data. All of them are girls. The first one is 18 months, presented at our clinic with the symptom of Cushing syndrome and abdomen mass. She also had hypertension leading to hypertrophy of her left ventricle. Her plasma and urine cortisol were elevated and the plasma ACTH were decreased. Imagine studies revealed neoplasm which size is 4.5 cm×3 cm×2 cm in her right adrenal gland. The second one is 4 year olds, complained the symptom of virilization: enlarged clitoris, man-like pubarche, facial hair and deep voice. The plasma testosterone concentration, plasma and urine cortisol concentration elevated. Ultrasound and CTscan revealed neoplasm which size is 5.5 cm×5 cm×3.5 cm on her left adrenal gland. Two of them were experienced to unilateral adrenalectomy and had characteristis of adrenal corticol adenoma on microscopic examination. On follow-up section, the first girl still had hypertension, the plasma and urine cortisol remained increasing. She was re-evaluated for recurrent tumor and was discovered sign of metastatis to the lung on chest CTscan. Unfortunaltely, her family refused to treament. Follow-up of the last patient showed that signs of virilization were suppressed. Serum testosterone levels dropped to normal after surgery, and remained normal. Two to six 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. Adrenalcortiacal tumor is needed to diagnosis and surgery as early as possible to prevent the effect of hormone over production. Althought our two cases were almost effectively treatmented by surgery but long term follow up is very importance to detect postoperative complications.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology