Patel Viral V, Shah Diva S, Raychaudhari Chandra R, Patel Keyuri B
Department of Radiodiagnosis, PramukhSwami Medical College and Shree Krishna Hospital, Karamsad, Gujarat, India.
Indian J Med Paediatr Oncol. 2010 Apr;31(2):65-8. doi: 10.4103/0971-5851.71659.
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.
肾上腺皮质癌（ACC）是一种罕见的恶性肿瘤，在儿童中尤为少见。总体发病率约为每年每百万人口2例。[1] 在儿童中，发病率为每年每百万人口0.3例，但巴西南部除外，那里的发病率为每年每百万人口3.4 - 4.2例。[2] 我们描述了一名6岁女孩，患有巨大的无功能转移性肾上腺皮质癌，其母亲自1周前偶然发现她的腹围增大。腹部超声显示右侧肾上腺有一个大肿瘤，伴有钙化和坏死。可见左肾窝空虚，右肾代偿性增大。计算机断层扫描（CT）显示右侧肾上腺有一个大的不均匀强化肿块，伴有钙化、坏死及肺转移。右侧肾上腺肿块的组织病理学报告显示为肾上腺皮质癌。该患者为IV期疾病，诊断后2个月死亡。
