ABSTRACT
Adrenocortical carcinoma is a very rare malignant tumour in children. It has bad prognosis and the treatment protocols are not well established. We report 2 cases of adrenocortical carcinoma in children. Case 1: A 30-month-old girl was hospitalized for clitorimegaly and pubic hair. The clinical examination found an abdominal mass with paraplegia. Abdominal ultrasonography found a heterogeneous calcified mass localised in the right suprarenal region. The diagnosis was confirmed by elevated urinary 17-ketosteroids. Complementary investigations showed pulmonary, medullar and vertebral metastasis. The treatment consisted of chemotherapy [Cisplatinum + Vepeside] with no efficacy. She died after 7 months follow up. Case 2: An 8-year-old boy was admitted for a pubic and axillary's hair, with acne and abdominal mass. The abdominal ultrasonography found a heterogeneous mass in the right suprarenal region. The diagnosis was confirmed by histology after complete resection. The patient was lost from follow-up and was seen one year later with abdominal mass associated to pulmonary and liver metastasis. He received only a palliative treatment. Comments: The adrenocortical carcinoma is a rare tumour in children [0.2% of solid tumours]. The malignant criteria and treatment protocols are not well established. The 5-year OS is 49%. The most important prognosis factor is total excision of the tumour