Cerebral Metastases from Adrenocortical Carcinoma in Children: a Case Report and Literature Review

Adrenocortical carcinoma (ACC) is a rare and aggressive tumor affecting mostly children younger than 5 years of age. In the staging and treatment of patients with ACC, a particular attention should be focused on possible isolated brain secondarisms, even if extremely rare. We report the case of a 3-year-old boy, with multiple cerebral secondarisms from an adrenocortical carcinoma associated with Beckwith-Wiedemann syndrome (BWS) due to imprinting control region 2 (IC2) hypomethylation, with unusual radiological characteristics. The adrenocortical tumor was diagnosed after the onset of precocious puberty at the age of 2 years and 11 months. Isolated cerebral metastases were already showed during the first cerebral magnetic resonance imaging (MRI) performed during pre-chemotherapy staging. The patient was treated with chemotherapy, after adrenal mass surgery and cerebral secondarisms biopsy. Multiple brain metastases from ACC are possible, even in children, as an isolated finding. Biopsy is mandatory in order to confirm or exclude metastases and to choose the best therapeutic approach.