Argenyi Z B, Van Rybroek J J, Kemp J D, Soper R T
Department of Pathology, University of Iowa, Iowa City 55240.
Am J Dermatopathol. 1988 Feb;10(1):59-67. doi: 10.1097/00000372-198802000-00008.
We present a case of a congenital angiomatoid malignant fibrous histiocytoma. This rapidly growing lesion, which was located in the subcutis of the left upper arm, was excised at the age of 8 1/2 months. The patient, a girl, was well and free of disease 10 months after surgical removal of the tumor. The tumor appeared grossly encapsulated. The gray-tan tissue contained cystic spaces filled with recent and organizing hemorrhages. Microscopically, the tumor was composed of solid masses of histiocyte- and fibroblast-like cells, inflammatory infiltrate, and multifocal irregular blood-filled spaces, which were predominantly devoid of endothelial cells. The tumor was studied immunohistochemically with antibodies specific for FVIII-related antigen, S-100 protein, epithelial membrane antigen, vimentin, desmin, alpha-1-antitrypsin, muramidase, laminin, and collagen type IV. Ulex europaeus lectin-I was also utilized. These studies, along with our ultrastructural findings, suggest that: (a) the tumor is composed of a mixture of mesenchymal cells; (b) an imperfect angiogenesis may be taking place, resulting in a wide spectrum of vascular structures; and (c) the cell of origin may be a pluripotent mesenchymal cell.
我们报告一例先天性血管瘤样恶性纤维组织细胞瘤。这个快速生长的病变位于左上臂皮下，在患儿8个半月大时被切除。该患者为一名女孩，在手术切除肿瘤10个月后情况良好，未患疾病。肿瘤肉眼可见有包膜。灰棕色组织中含有充满新鲜出血和机化性出血的囊腔。显微镜下，肿瘤由组织细胞样和成纤维细胞样细胞的实性团块、炎性浸润以及多灶性不规则充血间隙组成，这些间隙主要缺乏内皮细胞。用针对VIII因子相关抗原、S-100蛋白、上皮膜抗原、波形蛋白、结蛋白、α1抗胰蛋白酶、溶菌酶、层粘连蛋白和IV型胶原的抗体对肿瘤进行了免疫组织化学研究。还使用了欧洲荆豆凝集素-I。这些研究以及我们的超微结构发现表明：(a)肿瘤由间充质细胞混合组成；(b)可能正在发生不完全的血管生成，导致血管结构范围广泛；(c)起源细胞可能是多能间充质细胞。
