Corticoadrenaloma: A case report from the pediatric haemato-oncology and pediatric endocrinology departments at the Hassan II University Hospital of Fez

Abstract

Adrenocortical carcinoma is a rare tumor, representing 0.2% of pediatric cancers. Diagnosis is made on a combination of clinical, biological, radiological, and anatomopathological arguments. Treatment involves tumor excision surgery, sometimes supplemented by chemotherapy for forms with a high risk of recurrence. Prognosis is often reserved, with a non-negligible death rate. This report presents three cases of adrenocortical carcinoma treated at our institution between 2017 and 2023. The patients were three girls aged 5 years, 8 months, and 11 years, respectively. They were admitted for signs of hyperandrogenism and arterial hypertension. The tumor was left adrenal in all patients. The histological score was Severe Weiss 6 or 7. All patients underwent tumor resection, and they all benefited from this procedure. Despite chemotherapy in one patient, normalization of blood pressure figures postoperatively, two patients progressed to recurrence and subsequently died. The objective of this study is, through three observations, to describe the problem of the management of adrenocortical carcinoma (ACC) on the one hand, and to review the literature on the management and the prognosis of corticosteroid ACC in children.