Positive immunohistochemical staining for cytokeratins AE1 and AE3, epithelial membrane antigen (EMA), S-100, galectin-3 and brachyury as well as negative staining for glial fibrillary acidic protein (GFAP), carcinoembryonic antigen (CEA) and 5′-nucleotidase suggest a notochord lesion but do not provide clarity in differentiating between EP and chordoma [4]. Here, TBX1 is linked to chordoma.