2023 Volume 59 Issue 5 Pages 892-898
Pediatric adrenocortical carcinoma is a rare malignancy. We report a case of adrenocortical carcinoma of the adrenal cortex in a child who presented with acute abdomen and was diagnosed with signs of masculinization. The patient was a 7-year-old girl. She was transferred to our department after a contrast-enhanced CT scan of the abdomen revealed an 11 cm mass in the right retroperitoneum. She had tenderness in the right upper abdomen, palpable elastic induration under the right hypochondrium, and signs of masculinization such as adolescent male-like body odor, acne, and enlarged genitalia. We suspected adrenocortical carcinoma and performed a blood endocrine examination, which revealed a markedly high blood DHEA-S level of 24,680 ng/ml. We performed a right adrenal tumor resection, and histopathological analysis revealed adrenocortical carcinoma. Postoperatively, she received chemotherapy including mitotane. She has been under observation for 1 year and 4 months without recurrence. Pediatric adrenocortical carcinoma is often a functional tumor, and retroperitoneal tumors that present with characteristic symptoms such as masculinizing signs should be diagnosed and treated with this syndrome in mind.
