Abstract
Adrenocortical carcinoma (ACC) is a rare malignancy, especially in children. The overall
incidence is approximately 2 cases per million per year. [1] In children, the incidence
is 0.3 cases per million per year, except in southern Brazil where the incidence is
3.4-4.2 cases per million per year. [2] We describe a giant nonfunctioning metastasized
ACC in a 6-year-old girl who presented with a history of increasing abdominal girth
incidentally noticed by her mother since 1 week. Ultrasound abdomen showed a large
right suprarenal tumor with calcifications and necrosis. Empty left renal fossa and
compensatory enlarged right kidney were seen. Computed tomography (CT) scan revealed
a large heterogenously enhancing right suprarenal mass with calcification and necrosis
with pulmonary metastasis. Histopathology report from the right suprarenal mass revealed
an ACC. With a stage IV disease, the patient died after 2 months from diagnosis.
Keywords
Adrenocortical carcinoma - adrenocortical tumor - nonfunctioning
