Ewing's sarcoma/primitive neuroectodermal tumor of the chest wall

Ewing's sarcoma/primitive neuroectodermal tumor is the most common tumor of the chest wall in children and adolescents. It is extremely malignant with a high frequency of both metastatic spread and of local recurrence. Cure requires intensive therapy to control both distant and local disease. Surgery and high-dose radiotherapy can achieve equivalent local control; however, radiation is associated with the additional morbidities of second malignancy and a significant adverse impact on both cardiac and pulmonary function. The optimal therapeutic sequence is initial biopsy followed by induction chemotherapy with subsequent resection of the primary tumor. This approach will achieve the lowest incidence of tumor present at the margins of resection and, hence, need for postoperative radiotherapy. The chest wall is a rare site for tumors in children and adolescents. In a series reported from St Jude's Children's Research Hospital, chest wall tumors constituted only 1.8% of the solid childhood tumors. They are primarily mesenchymal in origin and the Ewing's sarcoma/primitive neuroectodermal tumors (PNET) predominate. This report concentrates on the later tumors. They are recognized to be extremely malignant, and cure in those who present with metastatic disease is very difficult to achieve. Recent advances in our understanding of their cytogenetic basis and optimal treatment are presented.