Background: Adrenocortical tumors (ACT) are rare in children. Mostly occurs in younger age, before 4 years and predominantly in girls. ACT represents 1.3% of all carcinomas in paediatric age group and 0.2% of all pediatric neoplasms.
Case report: 14 month old girl presented with signs of progressive hirsutism started first few months of life. She was referred to our clinic due to suspect diagnosis of virilizing CAH. Physical exams showed virilization in combination with signs of overproduction of other adrenal hormones: pronounced hirsutism (Ferriman Gallway score 25), clitoromegaly, hoarse voice, widespread acne on face. She has varus deformation of legs and a signs of pronounced rickets. She had accelerated growth. Her weight was over 98th percentile and height corresponded to 75th percentile for age and sex.
Lab tests: 17-OHP 3.51 ng/ml (0.16−1.02), Testosterone 7.06 ng/ml (0.03−0.32), BG- 75 mg/dl, insulin 16.51 MicU/ml, Cortisole 232 ng/ml (30−210). DHEAs −2.58 μg/ml (0.050.55). Advanced Bone age, more than 2 years compared to chronological was noted.Abdominal ultrasound revealed enlarged liver and spleen. MRT showed tumor size 5.5/4/4.7 cm in left upper quadrant in retroperitonium. Complete resection of encapsulated tumor without connection to adrenal was performed. No signs of local invasion or metastasis was found. Tumor was assessed locally, as well as by Institute of Pathology in Kiel, Germany as adrenal cortical tumor, immunohistochemicaly expressing Inhibin, Melanin A and Synaptophysin. Pathologist could not differentiate between adenoma and carcinoma.Postoperatively a brief episode of hypertension and polyuria occurred, therefore 24-hour blood pressure monitoring was conducted twice. The ambulatory BP monitoring recommended further. Testosterone was normalized in a week after surgery. Follow up visit 1.5 years later showed no signs of metastasis, change in appearance with decrease of virilisation and hirsutism to Ferriman Gallway score 9. Hormonal profile is normal, re-measured on multiple occasions. She follows up to monitor for possible metastasis every three month.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology