Ewing's sarcoma and extracranial primitive neuroectodermal tumors

The Ewing's family of tumors is comprised of bone and soft tissue, small round blue cell neoplasms of neuroectodermal origin defined by the chromosomal aberration t(11;22)(q24;q12), and closely related variants. Molecular methods now exist to facilitate diagnosis and to defect minimal residual disease. Multiagent chemotherapeutic regimens in concert with adequate local control yield improved survival rates in patients with localized disease at diagnosis. Patients with metastatic disease still suffer poor survival rates; programs attempting to cure metastatic patients with intensive therapy as facilitated by peripheral stem cell and autologous marrow rescue have shown some promise. Intensive regimens with and without rescue are being explored for high-risk patients.