Ewing's sarcoma is a very rare tumor which has, however, attracted muc
h oncological interest since the dramatic improvement of its prognosis
under chemotherapy. Its histogenesis has been discussed controversial
ly for a long time, including a possible origin in immature reticulum,
myogenous, endothelial and undifferentiated mesenchymal cells. Repeat
ed reports have also suggested a possible neuroectodermal genesis. Con
vincing arguments, however, have only been brought forward during rece
nt years, since it was found that Ewing's sarcoma and malignant periph
eral neuroectodermal tumor share a common chromosome translocation 11;
22. In the meantime this hypothesis has been strengthened by numerous
cell biological analyses. There seems to be no clear border between Ew
ing's sarcoma and malignant peripheral neuroectodermal tumors with def
inite neural differentiation. Histological differential diagnosis of E
wing's sarcoma has been improved by immunohistological methods. In mos
t cases, they can be distinguished from lymphoma (leucocyte common ant
igen, B and T markers) and embryonal rhabdomyosarcoma (muscle specific
actin, desmin) without problems. Apart from that, it is possible nowa
days to obtain antibodies against the MIC 2-protein, which is preferab
ly expressed in Ewing sarcoma. The diagnostics of Ewing's sarcoma and
the malignant peripheral neuroectodermal tumor have considerably been
enriched by the fact that the specific chromosome translocation t(11;2
2) can be proved molecular biologically. In contrast to the cytogeneti
c evidence, it is not necessary to establish cell cultures.