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肝血管肉瘤酷似肝窦阻塞综合征/静脉闭塞性疾病:病理-放射学相关性。

Hepatic angiosarcoma mimicking sinusoidal obstruction syndrome/venoocclusive disease: a pathologic-radiologic correlation.

机构信息

Department of Anatomic Pathology, Cleveland Clinic Foundation, OH 44195, USA.

出版信息

Ann Diagn Pathol. 2012 Aug;16(4):275-9. doi: 10.1016/j.anndiagpath.2011.10.009. Epub 2012 Feb 2.

Abstract

We present a case of a 63-year-old man with liver dysfunction and biopsy findings of venoocclusive disease (VOD) who, at autopsy, was discovered to have multifocal hepatic angiosarcoma. After double lung transplantation, he initially presented with signs of liver failure and portal hypertension resulting in recurrent high-volume ascites. Clinically, VOD was considered, and tacrolimus was discontinued, due to its known association with VOD. This, however, did not result in clinical improvement, and computed tomography eventually revealed the development of multiple low-attenuating hepatic lesions over the course of several months. Biopsies of the masses and background liver demonstrated changes most consistent with VOD, characterized by sinusoidal congestion affecting the centrilobular areas with associated hepatocyte atrophy and dropout. A reticulin stain highlighted deposition of reticulin fibers within the sinusoids and central veins. Scattered sinusoidal atypical cells were identified; however, a definitive diagnosis of malignancy was not possible. He eventually passed away because of complications of liver disease. At autopsy, there were multiple firm, red-brown masses identified throughout both hepatic lobes. Upon histologic review, the masses were shown to be angiosarcoma. Away from the tumor, the liver also demonstrated features of VOD. It is likely that the histologic appearance of VOD in the background liver probably represents secondary changes due to injury to the hepatic sinusoids by the primary malignancy. We conclude that it is necessary to consider the possibility of unsampled vascular malignancy when hepatic masses are identified on imaging and histology is consistent with VOD.

摘要

我们报告了 1 例 63 岁男性,因肝功能障碍和活检发现静脉阻塞性疾病(VOD),尸检时发现多灶性肝血管肉瘤。在双肺移植后,他最初表现为肝功能衰竭和门静脉高压的迹象,导致反复出现大量腹水。临床上考虑 VOD,并因与 VOD 相关而停用他克莫司。然而,这并没有导致临床改善,计算机断层扫描最终显示在几个月的时间内发展为多个低衰减肝病变。对肿块和背景肝的活检显示,变化最符合 VOD,特征为影响中心小叶区的窦状充血,伴有肝细胞萎缩和脱落。网状纤维染色突出了窦状隙和中心静脉内网状纤维的沉积。散在的窦状异型细胞被识别;然而,不能明确诊断为恶性肿瘤。他最终因肝病并发症去世。尸检时,在两个肝叶中发现多个坚硬的棕红色肿块。组织学检查显示这些肿块为血管肉瘤。远离肿瘤的肝脏也表现出 VOD 的特征。背景肝中 VOD 的组织学表现可能代表原发性恶性肿瘤对肝窦的损伤引起的继发性变化。我们得出结论,当影像学检查发现肝肿块,且组织学符合 VOD 时,有必要考虑未取样的血管恶性肿瘤的可能性。

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