Primitive neuroectodermal tumors of the chest wall

Abstract

Primitive neuroectodermal tumor (PNET) is a rare, highly malignant small-cell neoplasm that most often arises from the chest wall or paravertebral region. A patient is described from whom resection, intensive chemotherapy, and bone marrow transplant were unsuccessful. Eleven patients were treated between 1975 and 1985; there was only one long-term survivor (48 months) despite intensive multidisciplinary treatment. The median survival of 49 published cases was 9 months. Conservative resection minimizing disability, radiotherapy, and intensive chemotherapy remain valid options, but use of experimental agents as part of initial therapy seems justified. The role of autologous marrow transplant remains unclear.