TY - JOUR T1 - Adult Pleomorphic Rhabdomyosarcoma: A Multicentre Retrospective Study JF - Anticancer Research JO - Anticancer Res SP - 6213 LP - 6217 VL - 35 IS - 11 AU - JONATHAN NOUJAIM AU - KHIN THWAY AU - ROBIN L. JONES AU - AISHA MIAH AU - KOMEL KHABRA AU - RUPERT LANGER AU - BERND KASPER AU - IAN JUDSON AU - CHARLOTTE BENSON AU - ATTILA KOLLĂ€R Y1 - 2015/11/01 UR - http://ar.iiarjournals.org/content/35/11/6213.abstract N2 - 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. ER -