Adult Pleomorphic Rhabdomyosarcoma: A Multicentre Retrospective Study.
BACKGROUND
Pleomorphic rhabdomyosarcoma (RMS) is a rare sub-type of RMS. Optimal treatment remains undefined.


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.


CONCLUSIONS
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.
Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, U.K. john.c.njm@gmail.com.
Jonathan
J
Noujaim
Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, U.K.
Khin
K
Thway
Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, U.K.
Robin L
RL
Jones
Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, U.K.
Aisha
A
Miah
Research Data Management and Statistics Unit, Royal Marsden NHS Foundation Trust, London, U.K.
Komel
K
Khabra
Institute of Pathology, University of Bern, Bern, Switzerland.
Rupert
R
Langer
Sarcoma Unit, Interdisciplinary Tumour Center, Mannheim University Medical Center, University of Heidelberg, Mannheim, Germany.
Bernd
B
Kasper
Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, U.K.
Ian
I
Judson
Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, U.K.
Charlotte
C
Benson
Department of Medical Oncology, University Hospital Bern, Sarcoma Center Inselspital, Bern, Switzerland.
Attila
A
Kollàr
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology*; D009367:Neoplasm Staging; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology*; D015996:Survival Rate; D014750:Vincristine / Q000008:administration & dosage
D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D007069:Ifosfamide
Pleomorphic rhabdomyosarcoma; chemotherapy; multicenter study; prognosis