Haber L M, Truong L
Department of Pathology, Baylor College of Medicine, Houston, TX 77030.
Am J Surg Pathol. 1988 Oct;12(10):798-802.
Primary sarcomas of the aorta are rare; fewer than 30 cases have been reported. Among these, the majority are intraluminal and apparently intimal in origin. Extensive histochemical and electron-microscopic evaluation of these tumors has not previously been performed. We present a case of aortic intimal sarcoma in a 70-year-old man whose resected aorta showed multifocal, intimal tumor that appeared on light microscopy to be undifferentiated sarcoma. Electron microscopy was not helpful; however, immunohistochemical studies confirmed the endothelial nature of this neoplasm. The multifocal pattern of the tumor and the presence of intervening, atypical, proliferative endothelial cells suggests that endothelial dysplasia may have been a precursor lesion.
原发性主动脉肉瘤罕见，报道的病例不足30例。其中，大多数为腔内肿瘤，起源显然为内膜。此前尚未对这些肿瘤进行广泛的组织化学和电子显微镜评估。我们报告一例70岁男性的主动脉内膜肉瘤，其切除的主动脉显示多灶性内膜肿瘤，光镜下表现为未分化肉瘤。电子显微镜检查无帮助；然而，免疫组织化学研究证实了该肿瘤的内皮性质。肿瘤的多灶性模式以及其间存在的非典型增生内皮细胞提示内皮发育异常可能是前驱病变。
