Jonathan Noujaim1, Khin Thway2, Robin L Jones2, Aisha Miah2, Komel Khabra3, Rupert Langer4, Bernd Kasper5, Ian Judson2, Charlotte Benson2, Attila Kollàr6. 1. Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, U.K. john.c.njm@gmail.com. 2. Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, U.K. 3. Research Data Management and Statistics Unit, Royal Marsden NHS Foundation Trust, London, U.K. 4. Institute of Pathology, University of Bern, Bern, Switzerland. 5. Sarcoma Unit, Interdisciplinary Tumour Center, Mannheim University Medical Center, University of Heidelberg, Mannheim, Germany. 6. Department of Medical Oncology, University Hospital Bern, Sarcoma Center Inselspital, Bern, Switzerland.
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. Copyright
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. Copyright
Authors: Vishruth K Reddy; Varsha Jain; Robert J Wilson Ii; Lee P Hartner; Mark Diamond; Ronnie A Sebro; Kristy L Weber; Robert G Maki; Jacob E Shabason Journal: Sarcoma Date: 2021-03-13