Sakamoto Noriaki, Adachi Shuji, Monzawa Shuichi, Hamanaka Akihiro, Takada Yoshiki, Hunada Yasuhiro, Kotani Yoshikazu, Hanioka Keisuke
Department of Radiology, Hyogo Medical Center for Adults, Akashi City, Hyogo Prefecture, Japan.
J Thorac Imaging. 2005 Aug;20(3):236-8. doi: 10.1097/01.rti.0000158403.40711.e8.
Pulmonary epithelioid hemangioendotheliomas (PEH), also known as intravascular sclerosing bronchoalveolar tumor, is a rare vascular tumor of the lung common among young women. Primitive lumena lined by single cells is the characteristic pathologic feature. The endothelial nature of these cells is confirmed by positive staining with factor VIII and CD34. PEH usually presents as single or multiple pulmonary nodules. The present report describes high resolution CT (HRCT) findings of 2 cases with unusual manifestations of PEH. One case was a 54-year-old woman with multiple pulmonary nodules with irregular thickening of both the bronchovascular bundles and perilobular structures, representing intensive lymphangitic spread on HRCT. The other was an 18-year-old woman who had multiple minute peripheral nodules in the lungs bilaterally. These HRCT findings demonstrated the presence of tumor nodules in the lymphatic spaces, which is quite an unusual histologic presentation for this tumor. Both cases also showed hepatic lesions on abdominal CT; the former showing hypoattenuating masses with coarse calcifications and the latter showing multiple tiny calcifications in the hepatic parenchyma. Recognition of these features in the appropriate clinical setting may allow the clinician and the pathologist to consider this rare tumor.
肺上皮样血管内皮瘤（PEH），也称为血管内硬化性支气管肺泡瘤，是一种罕见的肺部血管肿瘤，常见于年轻女性。由单个细胞衬里的原始管腔是其特征性病理特征。这些细胞的内皮性质通过因子VIII和CD34阳性染色得以证实。PEH通常表现为单个或多个肺结节。本报告描述了2例具有不寻常表现的PEH的高分辨率CT（HRCT）表现。1例为54岁女性，有多个肺结节，支气管血管束和小叶周围结构均有不规则增厚，在HRCT上表现为广泛的淋巴管播散。另1例为18岁女性，双侧肺部有多个微小的外周结节。这些HRCT表现显示肿瘤结节存在于淋巴间隙，这对该肿瘤来说是相当不寻常的组织学表现。两例患者腹部CT均显示肝脏病变；前者表现为低密度肿块伴粗大钙化，后者表现为肝实质内多个微小钙化。在适当的临床背景下认识到这些特征，可能会使临床医生和病理学家考虑到这种罕见肿瘤。
