原发性肝平滑肌肉瘤：一例报告并文献复习
Primary Hepatic Leiomyosarcoma: a Case Report and Review of the Literature.
作者信息
Feretis Themistoklis, Kostakis Ioannis D, Damaskos Christos, Garmpis Nikolaos, Mantas Dimitrios, Nonni Afroditi, Kouraklis Gregory, Dimitroulis Dimitrios
机构信息
Second Department of Propedeutic Surgery, "Laiko" General Hospital, National and Kapodistrian University of Athens, Medical School, Athens, Greece.
First Department of Pathology, National and Kapodistrian University of Athens, Medical School, Athens, Greece.
出版信息
Acta Medica (Hradec Kralove). 2018;61(4):153-157. doi: 10.14712/18059694.2018.135.
BACKGROUND/AIM: Primary hepatic leiomyosarcoma is an extremely rare type of liver sarcoma with relatively poor prognosis, with about 50 cases having been reported in the literature. Potential origins of this tumor in the liver are the smooth muscle cells in the round ligament, intrahepatic blood vessels and intrahepatic bile ducts. There is no apparent sex predilection and there is a wide age range. The clinical presentation is not specific and the diagnosis depends on the expression of markers such as smooth muscle actin, desmin and vimentin by tumor cells.
PATIENTS AND METHODS
Herein, we present a case of a bulky primary hepatic leiomyosarcoma in a 68-year-old female patient. The patient underwent resection of the exophytic mass en block with the hepatic segments III and IVB.
CONCLUSION
Surgical resection is the most effective among the treatment options.
背景/目的：原发性肝平滑肌肉瘤是一种极其罕见的肝脏肉瘤类型，预后相对较差，文献报道约50例。该肿瘤在肝脏中的潜在起源是圆韧带、肝内血管和肝内胆管中的平滑肌细胞。无明显性别倾向，发病年龄范围广。临床表现不具特异性，诊断取决于肿瘤细胞中平滑肌肌动蛋白、结蛋白和波形蛋白等标志物的表达。
患者与方法
在此，我们报告一例68岁女性患者的巨大原发性肝平滑肌肉瘤病例。患者接受了将外生性肿块与肝段Ⅲ和ⅣB整块切除的手术。
结论
在治疗选择中，手术切除是最有效的。
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