[Primary neuroectodermal tumor (PNET): a rare highly malignant soft tissue tumor in children and young adults]

The PNET is an extremely malignant soft tissue neoplasm resulting from a balanced reciprocal translocation t (11; 22) (q24; q12). Treatment of the undifferentiated small cell tumor is carried out in compliance with the protocol of the soft tissue trail (CWS) from the German Society of Pediatric Oncology. Biopsy-proven diagnosis is followed by primary chemotherapy which leads in 95% of cases to remission. After excision of the remainder of the malignancy, an irradiation of the tumor site and two further sequences of chemotherapy are performed. Between 1986 and 1994, in cooperation with our pediatric and radiotherapeutical colleagues, we treated ten patients. In four patients (median age, 14 years) the PNET originated from the chest wall, in six patients from the paravertebral and retroperitoneal region. Five patients died after 20 months on average, while the remaining five patients are in full remission after 22, 37, 41, 42 and 82 months, respectively.