Adult Pleomorphic Rhabdomyosarcoma: A Multicentre Retrospective Study.

Noujaim, Jonathan; Thway, Khin; Jones, Robin L; Miah, Aisha; Khabra, Komel; Langer, Rupert; Kasper, Bernd; Judson, Ian; Benson, Charlotte; Kollár, Attila (2015). Adult Pleomorphic Rhabdomyosarcoma: A Multicentre Retrospective Study. Anticancer research, 35(11), pp. 6213-6217. International Inst. of Anticancer Research

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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.

Item Type:

Journal Article (Original Article)


04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Medical Oncology
04 Faculty of Medicine > Service Sector > Institute of Pathology

UniBE Contributor:

Langer, Rupert and Kollár, Attila


600 Technology > 610 Medicine & health
500 Science > 570 Life sciences; biology




International Inst. of Anticancer Research




Doris Haefelin

Date Deposited:

20 Jan 2016 13:54

Last Modified:

20 Jan 2016 13:54

PubMed ID:



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