小児副腎皮質癌の5自験例と本邦報告例の検討

概要

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Adrenocortical carcinoma is very rare disease in childhood. Here we reported our 5 cases of acrenocortical carcinoma and reviewed reported cases in Japan. Case 1 was Cushing syndrome and the other were adrenogenital syndrome (AGS). One was male and 4 were females. The distribution of age were from 5-month to 5-year 9-month-old. Their main clinical symptoms were abdominal tumor and hypertrophied clitolis. Serum and urinary hormonal analysis demonstrated high level of 17-OHCS (case 1) and 17-KS, DHEA (case 2, 4, 5). CT, angiography and 131I-rdosterol scintigraphy were effective measures to obtain the information of tumor. Complete tumor resection were performed in case 4, 5 and only these cases has been survived for more than two years postoperatively without the evidence of recurrence. In literature, only 50 cases were reported in Japan. Compared with adult cases, their clinical features were markedly differented (AGS 26, Cushing 9, combined 10, non-fuctioning 3 and unknown 2. Their prognosis were poor, however, recent advances of oncology and diagnostic methods has been enable to improve them.
日本小児外科学会の論文
1986-12-20