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Giant non-functioning adrenocortical carcinoma: A rare childhood tumor

Author(s): Patel Viral | Shah Diva | Raychaudhari Chandra | Patel Keyuri

Journal: Indian Journal of Medical and Paediatric Oncology
ISSN 0971-5851

Volume: 31;
Issue: 2;
Start page: 65;
Date: 2010;
Original page

Keywords: Adrenocortical carcinoma | adrenocortical tumor | nonfunctioning

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.

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