Plexiform Soft Tissue Tumor Composed Predominantly of Perineurial Fibroblasts (Perineurioma)

A 15-year-old girl presented with a small, indolent mass near the knee joint. Light microscopy revealed a peculiar myxoid plexiform tumor composed of cytologically bland cells. Interpretation of the initial biopsy material was controversial. Subsequent immunohistochemical studies revealed tumor cells to be strongly reactive for epithelial membrane antigen (EMA) and negative for S-100 protein. Ultrastructural studies revealed tumor cells with long, thin, bipolar cell processes and discontinuous basal laminae. They had no epithelial or histiocytoid features. Admixed among the tumor cells were Schwann cells, but they represented a rare and scattered component of the overall cell population. These features are most consistent with a so-called perineurioma and contrast with those of plexiform neurofibroma and traumatic neuroma (two lesions strongly positive for S-100 protein and showing a distinctive EMA immunoreactivity pattern with focal peripheral staining of neural bundles).