A Rare Paediatric Adrenocortical Carcinoma with Aggressive Clinical Course.

Adrenocortical carcinomas are among the rarest and most aggressive paediatric endocrine neoplasms. We report a case of a functional adrenocortical carcinoma in a 6-year and 7-month-old female who initially presented with hypertensive encephalopathy, later progressing to overt Cushing syndrome with refractory hypertension. Given the presence of distant metastases, the patient was commenced on neoadjuvant chemotherapy and mitotane. Genetic testing for TP53 to evaluate Li-Fraumeni syndrome was declined. Despite initial response to the treatment, the disease remained refractory, and the patient succumbed after seven months of therapy.