Adult pleomorphic rhabdomyosarcoma: a multicentre retrospective study
Background: Pleomorphic rhabdomyosarcoma (RMS) is a rare sub-type of RMS. Optimal treatment remains undefined. Patients and Methods: Between 1995 and 2014, 45 patients were diagnosed and treated in three tertiary sarcoma Centers (United Kingdom, Switzerland and Germany). Treatment characteristics an...
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| Main Authors: | , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
November 2015
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| In: |
Anticancer research
Year: 2015, Volume: 35, Issue: 11, Pages: 6213-6217 |
| ISSN: | 1791-7530 |
| DOI: | undefined |
| Online Access: | Verlag, teilw. kostenfrei, Volltext: http://dx.doi.org/undefined Verlag, teilw. kostenfrei, Volltext: http://ar.iiarjournals.org/content/35/11/6213 
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| Author Notes: | Jonathan Noujaim, Khin Thway, Robin L. Jones, Aisha Miah, Komel Khabra, Rupert Langer, Bernd Kasper, Ian Judson, Charlotte Benson and Attila Kollàr |
| Summary: | Background: Pleomorphic rhabdomyosarcoma (RMS) is a rare sub-type of RMS. Optimal treatment remains undefined. Patients and Methods: Between 1995 and 2014, 45 patients were diagnosed and treated in three tertiary sarcoma Centers (United Kingdom, Switzerland and Germany). Treatment characteristics and outcomes were analyzed. Results: The median age at diagnosis was 71.5 years (range=28.4-92.8 years). Median survival for those with localised (n=32, 71.1%) and metastatic disease (n=13, 28.9%) were 12.8 months (95% confidence interval=8.2-34.4) and 7.1 months (95% confidence interval=3.8-11.3) respectively. The relapse rate was 53.8% (four local and 10 distant relapses). In total, 14 (31.1%) patients received first line palliative chemotherapy including multi-agent paediatric chemotherapy schedules (n=3), ifosfamide-doxorubicin (n=4) and single-agent doxorubicin (n=7). Response to chemotherapy was poor (one partial remission with vincristine-actinomycin D-cyclophosphamide and six cases with stable disease). Median progression-free survival was 2.3 (range=1.2-7.3) months. Conclusion: Pleomorphic RMS is an aggressive neoplasm mainly affecting older patients, associated with a high relapse rate, a poor and short-lived response to standard chemotherapy and an overall poor prognosis for both localised and metastatic disease. |
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| Item Description: | Gesehen am 04.01.2018 |
| Physical Description: | Online Resource |
| ISSN: | 1791-7530 |
| DOI: | undefined |