title: Congenital angiomatoid malignant fibrous histiocytoma. A light-microscopic, immunopathologic, and electron-microscopic study.
abstract: 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.
authors.affiliation: Department of Pathology, University of Iowa, Iowa City 55240.
authors.forename: Z B
authors.initials: ZB
authors.lastname: Argenyi
authors.forename: J J
authors.initials: JJ
authors.lastname: Van Rybroek
authors.forename: J D
authors.initials: JD
authors.lastname: Kemp
authors.forename: R T
authors.initials: RT
authors.lastname: Soper
mesh_terms: D001808:Blood Vessels / Q000473:pathology; D005260:Female; D005347:Fibroblasts / Q000473:pathology; D006644:Histiocytes / Q000473:pathology; D018219:Histiocytoma, Benign Fibrous / Q000098:blood supply / Q000151:congenital* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D008854:Microscopy, Electron; D012878:Skin Neoplasms / Q000098:blood supply / Q000151:congenital* / Q000473:pathology