Parachordoma: a clinicopathologic and immunohistochemical study of four cases of an unusual soft tissue neoplasm.

Four soft tissue tumors corresponding with the previously reported parachordoma are described. Three of the patients were men, and one was a woman, and their ages ranged from 14 to 53 years (mean age, 29). The tumors were located either superficially or within muscle, and, in one case, involved a tendon. Histologically, the tumors displayed whorls, nests, and pseudoglandular cords of uniform polygonal cells with eosinophilic, vacuolated cytoplasm, in a focally myxoid stroma. Transitions were seen between fascicles of ovoid-spindled cells, with scanty cytoplasm in a fibrous stroma, and, in one case, whorls of bland spindly cells were also present. Electron microscopy of one case showed cells with short interdigitating microvilli and ill-defined junctions. The principal cells in all cases were positive for S100 protein, Leu-7, keratin (CAM5.2), and epithelial membrane antigen (EMA). All tumors were negative with antibody AE1 and with antibodies to cytokeratins CK7 and CK19. No tumor displayed immunoreactivity for carcinoembryonic antigen (CEA), muscle specific actin (MSA), smooth muscle actin (SMA), desmin, glial fibrillary acid protein (GFAP), CD31, or CD34. Parachordoma appears to be an entity with clinical and pathological differences from chordoma, which has a different cytokeratin profile, behaves in a more aggressive fashion, and can dedifferentiate. The differential diagnosis includes myxoid chondrosarcoma, myoepithelial cell tumor, ossifying fibromyxoid tumor, and chondroid lipoma.