Yang P, Hirose T, Hasegawa T, Seki K, Hizawa K
First Department of Pathology, University of Tokushima School of Medicine, Japan.
Am J Dermatopathol. 1995 Apr;17(2):179-84. doi: 10.1097/00000372-199504000-00011.
We present two cases of aneurysmal fibrous histiocytoma of the skin on the lower extremities of a 41-year-old man and a 23-year-old woman. Both the tumors appeared clinically as cutaneous nodules with a recent history of rapid growth and showed microscopically a unique formation of blood-filled tissue spaces and a storiform proliferation of histiocyte-like and fibroblast-like cells in a capillary-rich stroma. Such clinical and histologic features fit the original description of this entity. Immunohistochemically the tumor cells of both cases were immunoreactive for factor XIIIa, Mac 387, and vimentin and gave negative results for factor VIII-related antigen, desmin, actin, and S-100 protein. Ultrastructurally the tumors were composed mainly of siderosome-containing histiocyte-like cells, fibroblast-like cells, and intermediate cells and lacked prominent proliferation of endothelial cells. Thus, the ultrastructural findings agreed with the immunophenotypes expressed by the tumor cells, supporting the fibrohistiocytic origin of this lesion. In view of the recent rapid growth, the presence of hemorrhagic pseudocysts, the extravasation of erythrocytes, and the high vascularity of the present tumors, we emphasize the importance of distinguishing this lesion from angiomatoid fibrous histiocytoma and cutaneous malignancies of mesenchymal origin.
我们报告了两例皮肤动脉瘤样纤维组织细胞瘤，分别发生于一名41岁男性和一名23岁女性的下肢。这两个肿瘤在临床上均表现为皮肤结节，近期有快速生长史，显微镜下显示有独特的充满血液的组织间隙形成，以及在富含毛细血管的间质中组织细胞样和成纤维细胞样细胞呈车辐状增生。这些临床和组织学特征符合该实体的最初描述。免疫组化方面，两例肿瘤细胞对因子XIIIa、Mac 387和波形蛋白呈免疫反应，而对因子VIII相关抗原、结蛋白、肌动蛋白和S-100蛋白呈阴性结果。超微结构上，肿瘤主要由含铁血黄素小体的组织细胞样细胞、成纤维细胞样细胞和中间细胞组成，缺乏内皮细胞的显著增生。因此，超微结构结果与肿瘤细胞表达的免疫表型一致，支持该病变的纤维组织细胞起源。鉴于目前肿瘤近期的快速生长、出血性假囊肿的存在、红细胞外渗以及高血管化，我们强调将此病变与血管瘤样纤维组织细胞瘤和间叶源性皮肤恶性肿瘤相鉴别的重要性。
