Adult pleomorphic rhabdomyosarcoma

• Verfasst von: Noujaim, Jonathan [VerfasserIn]
• Verfasst von: Kasper, Bernd [VerfasserIn]
• Titel: Adult pleomorphic rhabdomyosarcoma
• Titelzusatz: a multicentre retrospective study
• Verf.angabe: Jonathan Noujaim, Khin Thway, Robin L. Jones, Aisha Miah, Komel Khabra, Rupert Langer, Bernd Kasper, Ian Judson, Charlotte Benson and Attila Kollàr
• E-Jahr: 2015
• Jahr: November 2015
• Umfang: 5 S.
• Fussnoten: Gesehen am 04.01.2018
• Titel Quelle: Enthalten in: Anticancer research
• Ort Quelle: Kapandriti, Attiki, Greece : International Institute of Anticancer Research, 2004
• Jahr Quelle: 2015
• Band/Heft Quelle: 35(2015), 11, Seite 6213-6217
• ISSN Quelle: 1791-7530
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: chemotherapy
• Sach-SW: multicenter study
• Sach-SW: Pleomorphic rhabdomyosarcoma
• Sach-SW: prognosis
• K10plus-PPN: 1566864690

Abstract

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.