[Primary neuroectodermal tumor (PNET): a rare highly malignant soft tissue tumor in children and young adults]
Autor: | T, Zimmermann, R, Blütters-Sawatzki, H, Christiansen, C, Kelm, W, Padberg |
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Jazyk: | němčina |
Rok vydání: | 1996 |
Předmět: |
Adult
Male Patient Care Team Adolescent Chromosomes Human Pair 11 Chromosomes Human Pair 22 Soft Tissue Neoplasms Combined Modality Therapy Translocation Genetic Survival Rate Chemotherapy Adjuvant Child Preschool Humans Neuroectodermal Tumors Primitive Female Radiotherapy Adjuvant Child Follow-Up Studies Neoplasm Staging |
Zdroj: | Langenbecks Archiv fur Chirurgie. Supplement. Kongressband. Deutsche Gesellschaft fur Chirurgie. Kongress. 113 |
ISSN: | 0942-2854 |
Popis: | 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. |
Databáze: | OpenAIRE |
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