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Clinical Sarcoma Research

, 3:14

First Online: 26 November 2013Received: 03 October 2013Accepted: 22 November 2013DOI: 10.1186-2045-3329-3-14

Cite this article as: Wong, H.H., Hatcher, H.M., Benson, C. et al. Clin Sarcoma Res 2013 3: 14. doi:10.1186-2045-3329-3-14


BackgroundDesmoplastic small round cell tumour DSRCT is a rare but frequently fatal sarcoma, and many of its characteristics still require further clarification.

MethodsWe retrospectively analysed 41 patients treated at or referred to two regional referral centres in the UK between 1991 and 2012. A review of the current literature was also performed.

ResultsThe median age of presentation was 27 years range 16 to 45 years, with a male-to-female ratio of 3:1. Ninety percent of patients had disease in the abdomen. The median size of the presenting tumour was 13 cm range 3.5 to 23 cm, and 80% had metastatic disease at diagnosis, mainly in the liver 33% and lungs 21%. Time-to-progression TTP was 3.9, 2.3 and 1.1 months after first-, second- and third-line chemotherapy, respectively. First-line treatment with VIDE chemotherapy appeared to confer the longest TTP median 14.6 months. Ifosfamide and doxorubicin resulted in TTP of >3.8 months when used in any-line setting. Eleven patients received targeted agents as part of a clinical trial. After a median follow-up of 14 months, the overall median survival MS was 16 months. There was no difference in MS with regards to age, gender, or size of the presenting tumour. Patients with extra-abdominal disease survived longer compared to those with tumours in the abdomen all still alive vs MS of 15 months; P = 0.0246. Patients with non-metastatic intra-abdominal disease who underwent surgery had an MS of 47 months 16 months for those who did not have surgery; P = 0.0235. Radiotherapy for locoregional control in patients with metastatic intra-abdominal DSRCT was associated with longer survival MS of 47 vs 14 months; P = 0.0147.

ConclusionsDSRCT is a rare but often fatal disease that mainly affects younger male patients. Those with intra-abdominal DSRCT have a poorer prognosis, although surgical resection for localised disease and radiotherapy in the metastatic setting are associated with improved survival. A patient’s age, gender and size of presenting tumour do not have prognostic significance.

KeywordsDesmoplastic small round cell tumour Sarcoma Prognosis Survival Treatment Electronic supplementary materialThe online version of this article doi:10.1186-2045-3329-3-14 contains supplementary material, which is available to authorized users.

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Autor: Han Hsi Wong - Helen M Hatcher - Charlotte Benson - Omar Al-Muderis - Gail Horan - Cyril Fisher - Helena M Earl - Ian J


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