Advanced Pediatric Adrenocortical Carcinoma: A Case Report

Abstract

Adrenocortical carcinoma (ACC) is rare in children. The prognosis of ACC is poor, especially in advanced disease stage. Herein we report a pediatric case of advanced ACC with pulmonary metastasis.
An 8-year-old boy with large abdominal mass was admitted to our hospital. Pubic hair and penile hypertrophy were observed on admission. A CT scan demonstrated a large tumor in the left adrenal grand and 3 lung nodules in the right upper lobe, right lower lobe, and left lower lobe. After tumor biopsy, he was diagnosed with ACC stage IV. Thus, he underwent resection of the left adrenal gland tumor. After the operation, he received adjuvant chemotherapy including mitotane, an antiadrenal agent for ACC. The lung metastases remained unchanged and new lesions were not found during his clinical course. He underwent resection of the right and left lung metastases 7 and 16 months after the primary operation, respectively. He was free from recurrence as of 28 months after the admission.
While there is no established treatment protocol for advanced ACC in children, complete resection of the tumor with adjuvant chemotherapy including mitotane would be effective.