Extraosseous Ewing's sarcoma versus primitive rhabdomyosarcoma: diagnostic criteria and clinical correlation

A light and electron microscopic study of 51 cases of Ewing's sarcoma of bone (ESB) and 33 soft tissue sarcomas (carrying a variety of light microscopic diagnoses, including primitive rhabdomyosarcoma) in children and young adults was performed to clarify the similarities and differences among these tumors. Ultrastructural criteria were developed to evaluate the neoplasms. Remarkable ultrastructural uniformity was found in the cases of ESB. In contrast, the soft tissue sarcomas could be divided into two distinct groups on the basis of the ultrastructural criteria: those closely resembling primitive areas of otherwise differentiated rhabdomyosarcomas, and those indistinguishable from ESB. It is proposed that the diagnosis of soft tissue Ewing's sarcoma be reserved for lesions identical to ESB by both light and electron microscopy. The first group of sarcomas may be histogenetically related to rhabdomyosarcoma and should be distinguished from extraosseous Ewing's sarcoma, as their clinical behavior appears to be quite different.