Miettinen M, Gannon F H, Lackman R
Department of Pathology and Cell Biology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107.
Ultrastruct Pathol. 1992 Sep-Oct;16(5):577-86. doi: 10.3109/01913129209061549.
A soft tissue tumor in the leg of a 67-year-old woman is described. This large tumor below the knee area infiltrated extensively the deep and superficial soft tissues but did not involve the bones. The tumor cells formed nodules resembling the architecture seen in chondroid tumors and chordoma. The tumor cells were often vacuolized, and there was extracellular myxoid matrix similar to that in chordoma or myxoid chondrosarcoma. Immunohistochemistry showed keratins 8 and 19, epithelial membrane antigen, and vimentin in most tumor cells, and there was also S-100 protein positivity in a number of tumor cells. Electron microscopy showed desmosomelike cell junctions and bundles of intermediate filaments resembling those seen in many epithelial neoplasms. Thus the tumor resembled chordoma in many respects. Because clinically no other primary tumor was found, this tumor is probably a chordomalike primary soft tissue sarcoma different from typical extraskeletal myxoid chondrosarcoma or chordoid sarcoma.
描述了一名67岁女性腿部的软组织肿瘤。这个位于膝下区域的大肿瘤广泛浸润深部和浅部软组织，但未累及骨骼。肿瘤细胞形成结节，类似于软骨样肿瘤和脊索瘤中的结构。肿瘤细胞常呈空泡状，并有与脊索瘤或黏液样软骨肉瘤中相似的细胞外黏液样基质。免疫组化显示大多数肿瘤细胞中有角蛋白8和19、上皮膜抗原及波形蛋白，并且许多肿瘤细胞中也有S-100蛋白阳性。电镜显示有类似桥粒的细胞连接和类似于许多上皮性肿瘤中所见的中间丝束。因此，该肿瘤在许多方面类似于脊索瘤。由于临床上未发现其他原发性肿瘤，该肿瘤可能是一种不同于典型骨外黏液样软骨肉瘤或脊索样肉瘤的类脊索瘤原发性软组织肉瘤。
