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范可尼贫血患者的肝纤维板层癌。

Fibrolamellar carcinoma of the liver in a patient with Fanconi anemia.

作者信息

LeBrun D P, Silver M M, Freedman M H, Phillips M J

机构信息

Department of Pathology, Hospital for Sick Children, Toronto, Ontario, Canada.

出版信息

Hum Pathol. 1991 Apr;22(4):396-8. doi: 10.1016/0046-8177(91)90088-7.

Abstract

A 9-year-old boy with Fanconi anemia treated with oxymethalone, a synthetic androgen, died of intracerebral hemorrhage. At autopsy, the liver contained several adenomas and a large fibrolamellar hepatocellular carcinoma, as well as phlebectatic peliosis hepatis. The 11 previously reported cases of hepatocellular carcinoma in Fanconi anemia were not, apparently, of the fibrolamellar type, which has a better prognosis, occurs in children of both sexes, and generally is not associated with cirrhosis. The malignant potential of primary liver tumors associated with Fanconi anemia as well as the nature of their relationship to Fanconi anemia and to anabolic steroid therapy is discussed.

摘要

一名患有范可尼贫血的9岁男孩接受了合成雄激素羟甲烯龙治疗,死于脑出血。尸检发现,肝脏中有多个腺瘤、一个大的纤维板层型肝细胞癌以及肝静脉扩张性紫癜。之前报道的11例范可尼贫血合并肝细胞癌的病例显然并非纤维板层型,该型预后较好,男女儿童均可发生,且一般与肝硬化无关。本文讨论了范可尼贫血相关原发性肝肿瘤的恶性潜能及其与范可尼贫血和合成代谢类固醇治疗的关系本质。

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