caa a22 4500 463180097 CHVBK 20180406164836.0 cr unu---uuuuu 170326e20071001xx s 000 0 eng 10.1007/s00381-007-0392-8 doi (NATIONALLICENCE)springer-10.1007/s00381-007-0392-8 Sarcomas in children with neurofibromatosis type 1—poor prognosis despite aggressive combined therapy in four patients treated in a single oncological institution [Elektronische Daten] [Ewa Bien, Teresa Stachowicz-Stencel, Danuta Sierota, Katarzyna Polczynska, Anna Szolkiewicz, Joanna Stefanowicz, Elzbieta Adamkiewicz-Drozynska, Piotr Czauderna, Wojciech Kosiak, Miroslawa Dubaniewicz-Wybieralska, Ewa Izycka-Swieszewska, Anna Balcerska] Objects: Patients with neurofibromatosis type 1 (NF1) are predisposed to developing soft tissue sarcomas (STS). Materials and methods: We report on four cases of STS diagnosed in locally advanced, unresectable stages in children with NF1 (three girls, one boy; age = 8months-14years). All patients received protocols for STS: Cooperative Weichteilsarkomstudie 91, 96 and 2002. One patient with limb rhabdomyosarcoma entered complete remission but developed late metastatic relapse and died of progression despite complete excision and autologous bone marrow transplantation. The other patient with bladder rhabdomyosarcoma died of neutropenia-related sepsis without remission. Patients with malignant peripheral nerve sheet tumour and malignant triton tumour located in the pelvis did not respond to therapy. One of them died of disease progression, while the other is disease-free 6years post-therapy after mutilating tumour resection. Conclusion: STS in NF1 seem to display poor prognosis in spite of combined therapy; thus, children with NF1 should remain under detailed control of the oncologist. Springer-Verlag, 2007 Neurofibromatosis type 1 nationallicence Neurogenic sarcomas nationallicence Non-neurogenic sarcomas nationallicence Response to therapy nationallicence Prognosis nationallicence Children nationallicence Bien Ewa Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Stachowicz-Stencel Teresa Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Sierota Danuta Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Polczynska Katarzyna Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Szolkiewicz Anna Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Stefanowicz Joanna Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Adamkiewicz-Drozynska Elzbieta Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Czauderna Piotr Department of Paediatric Surgery, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Kosiak Wojciech Department of Paediatric Nephrology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Dubaniewicz-Wybieralska Miroslawa Department of Radiology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Izycka-Swieszewska Ewa Department of Anatomopathology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Balcerska Anna Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut Child's Nervous System Springer-Verlag 23/10(2007-10-01), 1147-1153 0256-7040 23:10<1147 2007 23 381 https://doi.org/10.1007/s00381-007-0392-8 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00381-007-0392-8 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Bien Ewa Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 700 1- Stachowicz-Stencel Teresa Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 700 1- Sierota Danuta Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 700 1- Polczynska Katarzyna Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 700 1- Szolkiewicz Anna Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 700 1- Stefanowicz Joanna Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 700 1- Adamkiewicz-Drozynska Elzbieta Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 700 1- Czauderna Piotr Department of Paediatric Surgery, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 700 1- Kosiak Wojciech Department of Paediatric Nephrology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 700 1- Dubaniewicz-Wybieralska Miroslawa Department of Radiology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 700 1- Izycka-Swieszewska Ewa Department of Anatomopathology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 700 1- Balcerska Anna Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University, 7 Debinki Street, 80-211, Gdansk, Poland aut NATIONALLICENCE 773 0- Child's Nervous System Springer-Verlag 23/10(2007-10-01), 1147-1153 0256-7040 23:10<1147 2007 23 381 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer