Extraskeletal myxoid chondrosarcoma is a rare soft-tissue sarcoma of the extremities that is considered to be indolent, but yet it has a capacity for multiple recurrences and lung metastasis. In a 23-year period, 10 cases of this rare tumor were reported in this institute. The authors studied the clinicopathologic features, including histochemical and immunohistochemical reactions, of these 10 cases, with a view to evaluate whether any constant pattern emerged. The histologic features include a lobular or nodular growth pattern, periodic acid-Schiff's stain positivity, diastase sensitivity, alcian blue positivity with and without pretreatment by testicular hyaluronidase, lack of epithelial markers, and positivity for S-100 protein and vimentin. The purpose of this article is to stress that the above-mentioned features are of immense help in delineating this rare tumor from other tumors associated with myxoid and chondroid differentiation. The authors did not find any consistent relationship between site, cellularity, and development of lung metastasis. Int J Surg Pathol 5(3/4): [77][78][79][80][81][82] 1997