ESPE Abstracts

Adrenocortical carcinoma is very rare in children, with annual incidence of 0.21 case per million patient younger than 20 years. At this article I reported two patients. First case was A 16-month-old girl presented with 3 months history of increased appetite and rapid weight gaining, appearance of hair at pubic area for 2 months and acne on face for one-month duration. Parents are non-consanguineous, had no endocrine disorders or tumors. On examination: chubby baby, cushingoid faces, three acne on the forehead, not dysmorphic. Weight and height were above 97th centile. Tanner stage: B1PH3. Enlarged clitoris, length: 2 cm. Investigations: elevated serum cortisol level: 789 nmol/l, low ACTH: < 1 pg/ml, elevated DHEAS: 927 mC/dl (Normal: 16-96 mC/dl) and high testosterone: 5.46 nmol/l. Renal profile and electrolytes: normal. Bone age: advanced= 2 years +8 months. CT abdomen: large left sided suprarenal mass with dimensions of 2.4 x 3.5 x 3.2 cm with no signs of calcifications, infiltration and no metastasis to liver or lungs. Multiple paraaortic lymph nodes were noted. Due to financial concerns of parents, the patient was referred to other hospital, at which left adrenalectomy done. Clinicopathological classification was corresponding with a malignant adrenocortical carcinoma. Adrenolytic therapy was given using mitotane. Father refused to do genetic study to rule out P53 germline mutation. Currently, the patient is on replacement dose of hydrocortisone. The second case is a 26-month-old boy brought by parents concerned of hair appearance at pubic area associated with smell of adult sweat for 2 months duration. Parents are non-consanguineous. Mother had total thyroidectomy 13 years ago for ??Papillary thyroid carcinoma followed by radioactive Iodine therapy. On examination: chubby baby with cushingoid faces. Both weight and height were above 97th centile. No acne, not dysmorphic. Tanner stage: T1PH3, Stretch penile length: 7 cm, width: 1.2 cm. Investigations: elevated serum cortisol: 650 nmol/l, low ACTH : < 1 pg/ml, high DHEAS : 221 mC/dl (Normal: 11-120 mC/dl) and elevated testosterone: 22.4 nmol/l. Renal profile and electrolytes: normal. Bone age: advanced = 4 years. CT abdomen : well defined mass at Right Adrenal gland measuring 2.5 x 2.7 x 2 cm. Due to financial concerns of parents, the patient was referred to other hospital, at which right adrenalectomy done. Clinicopathological classification was corresponding with a benign adrenocortical adenoma. The patient was on replacement hydrocortisone therapy, weaned later gradually. Currently patient is off treatment.