交差性片側肥大をともなった肝芽腫の1例

概要

論文の詳細を見る
The association of malignant neoplasms with certain congenital disorders is well known. A female infant with liver tumor and hemihypertrophy is reported. The patient was born at term after a normal pregnancy. Left hemihypertrophy and right macroglossia were noted at birth. At the age of six months, a large palpable mass in the right upper portion of abdomen was noted with high serum levels of alpha-fetoprotein. An arteriogram revealed a large mass in the right lobe of the liver. Tumor blood supply was from the superior mesenteric artery and a malignant liver tumor was suspected. At operation, the tumor was totally excised by right hepatectomy : The diagnosis was hepatoblastoma, en-bryonal type, confirmed by histopathological examination. Nine weeks after surgery, the patient developed severe thrombocytopenia. The thrombocytopenia was treated with steroids and gamma-globulin, resulting in rapid recovery. The increased tendency of chirdren with congenital hemihypertrophy to develop Wilms'tumor or adrenal neoplasm is well described. Association with hepatoblastoma or hemangioma is not so well recognized. This is the twelfth known case with the association In patients with hemihypertrophy we currently recommend not only excretory urograms but also liver ultrasonography at intervals of six months.
日本小児外科学会の論文
1990-08-20