Pediatric adrenocortical tumor in a 17-month-old girl: Diagnostic and therapeutic challenges in a rare case of virilization

BACKGROUND: Adrenocortical tumors (ACTs) are extremely uncommon in children. These tumors predominantly secrete hormones, leading to significant clinical manifestations, including peripheral precocious puberty and virilization. The diagnostic approach, which includes clinical assessment, laboratory tests, imaging, and histopathological evaluation, presents challenges in differentiating benign from malignant ACTs. CASE REPORT: We describe a 17-month-old girl who underwent pubic hair growth over a four-month period. Imaging tests revealed a solid-cystic mass in the right adrenal gland, which raised suspicion of malignancy. Histopathological analysis confirmed an ACT with moderate to high nuclear atypia and lymphovascular invasion following the patient's right adrenalectomy. Adjuvant treatment with doxorubicin, etoposide, and cisplatin was started because of the tumor's intermediate malignancy score and lymphovascular invasion. CONCLUSION: This case report emphasizes how difficult it is to diagnose and treat pediatric ACTs, underscoring the significance of early identification, thorough evaluation, and individualized care. Optimizing results requires a multidisciplinary approach and continuous follow-up due to the tumor's rarity and potential malignancy.