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
Full text not available from this repository.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) |
|---|---|
Division/Institute: |
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, Kollár, Attila |
Subjects: |
600 Technology > 610 Medicine & health 500 Science > 570 Life sciences; biology |
ISSN: |
0250-7005 |
Publisher: |
International Inst. of Anticancer Research |
Language: |
English |
Submitter: |
Doris Haefelin |
Date Deposited: |
20 Jan 2016 13:54 |
Last Modified: |
05 Dec 2022 14:51 |
PubMed ID: |
26504053 |
URI: |
https://boris.unibe.ch/id/eprint/74598 |
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