Ossifying Fibromyxoid Tumor: Modified Myoepithelial Cell Tumor? Report of Three Cases with Immunohistochemical and Electron Microscopic Studies

Abstract

Ossifying fibromyxoid tumors (OFMT) are rare soft tissue tumors of uncertain histogenesis and clinical behavior. Since Enzinger, Weiss, and Liang first described 59 examples in 1989 (Am Surg Pathol. 13:817–827), approximately 150 cases have been reported. Their clinicopathologic features are fairly well characterized and their histogenesis remains unknown. Three examples of soft tissue tumors with typical histopathologic characteristics of OFMT were studied: case 1, a 43-year-old female with a 2.5-cm tumor of the back; case 2, a 56-year-old man with an 8-cm thigh mass; and case 3, an 81-year-old female with a 13.5-cm buttock tumor. For immunohistochemistry, formalin-fixed, paraffin-embedded tissue sections were stained with antibodies against cytokeratin, smooth muscle actin, desmin, vimentin, S-100 protein, EMA, and collagen type IV using standard ABC-peroxidase methods. For electron microscopy, tissue samples fixed in EM-grade buffered formalin were processed according to routine methods. Immunohistochemistry showed that the tumor cells were positive for vimentin and S-100 protein in all 3 cases. Stains for collagen type IV revealed diffusely positive staining in the stroma with a tendency for stronger staining around the cell borders in 2 out of 3 cases. Desmin was positive in one and actin was positive in one other case. By electron microscopy, tumor cells were characterized by centrally located round to oval nuclei with varying amounts of cytoplasm containing scanty cytoplasmic organelles. There were rare profiles of rough-surfaced endoplasmic reticulum (RER) and rare mitochondria with areas of condensed intermediate filaments. No tonofilaments or actin filaments were present. There were multiple short web-like processes, some of which were attached to that of neighboring cells by primitive cell junctions. In all 3 cases, lesional cells showed external lamina (EL), which was abundant in case 1, forming redundant scrolls frequently. In case 2, EL was less prominent and incomplete, and interrupted portions of EL were present only along the periphery of cell columns or nests bordering the stroma. In case 3, which behaved as a malignant tumor, the tumor cells were less differentiated spindle cells with primitive cellular features, and EL was rarely found along the short span of tumor cell borders. In this study, tumor cells in OFMT were polygonal to stellate often with multiple short cytoplasmic processes. The tumor cells were found to form cell clusters attached by primitive intercellular junctions between cytoplasmic processes forming intercellular bridges. The cell borders facing the stroma around cell clusters tended to be flat and had incomplete EL, while no EL was present along the cell borders facing the inner aspect of cell clusters. These ultrastructural findings together with immunophenotypic expression of S-100 protein presented closer resemblance to those of modified myoepithelial cells in pleomorphic adenomas of salivary glands and skin appendages rather than peripheral nerve sheath tumors. The authors conclude that these findings render more support to the hypothesis of myoepithelial histogenesis of OFMT. They also conclude that ultrastructural study not only helps accurate diagnosis, but also may aid in predicting malignant behavior by the degree of deviation from the typical examples of OFMT.

KEYWORDS:

• histogenesis
• immunohistochemistry
• myoepithelial cells
• ossifying fibromyxoid tumor
• ultrastructure