Scoazec J Y, Lamy P, Degott C, Reynes M, Feldmann G, Bismuth H, Benhamou J P
Laboratoire de Biologie Cellulaire, CHU Bichat, Paris, France.
Gastroenterology. 1988 Jun;94(6):1447-53. doi: 10.1016/0016-5085(88)90685-3.
Five cases of epithelioid hemangioendothelioma of the liver are reported. This unusual type of vascular tumor is often difficult to diagnose; its angiogenic nature is not overt at the radiologic level and may be overlooked by conventional histologic examination. At imaging procedures, epithelioid hemangioendothelioma of the liver presents as multiple focal hypovascular areas, disseminated in both lobes. The combination of these imaging abnormalities with a peculiar set of demographic and clinical features, including young age, good general condition, and progressive course, is suggestive. However, the final diagnosis can be established only by histologic examination of appropriate material collected by guided liver biopsies and may be helped by immunohistochemistry and ultrastructural examination. The accurate diagnosis of epithelioid hemangioendothelioma of the liver is of clinical and therapeutic relevance. In view of its favorable clinical course, orthotopic liver transplantation may be considered for the treatment of this tumor when intrahepatic dissemination contraindicates partial hepatectomy and extrahepatic extension, excluded by exploratory laparotomy, is absent.
本文报告了5例肝脏上皮样血管内皮瘤。这种不常见的血管肿瘤通常难以诊断；其血管生成特性在放射学层面并不明显，可能会被传统组织学检查忽略。在影像学检查中，肝脏上皮样血管内皮瘤表现为多个局灶性低血供区域，散布于两叶。这些影像学异常与一组特殊的人口统计学和临床特征相结合，包括年轻、一般状况良好和病程进展，具有提示意义。然而，最终诊断只能通过经皮肝穿刺活检获取的合适材料进行组织学检查来确立，免疫组织化学和超微结构检查可能会有所帮助。肝脏上皮样血管内皮瘤的准确诊断具有临床和治疗意义。鉴于其良好的临床病程，当肝内播散排除了部分肝切除术且经剖腹探查排除肝外转移时，原位肝移植可考虑用于治疗该肿瘤。
