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Indian Journal of Medical and Paediatric Oncology
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CASE REPORT
Year : 2010  |  Volume : 31  |  Issue : 1  |  Page : 24-27

Intra-abdominal desmoplastic small round cell tumor: Presentation of four cases and review of the literature


1 Department of Nuclear Medicine, Radiotherapy and Oncology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kelantan, Malaysia
2 Department of Pathology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kelantan, Malaysia

Correspondence Address:
Biswa Mohan Biswal
Department of Nuclear Medicine, Radiotherapy and Oncology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-5851.68849

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Desmoplastic small round cell tumor (DSRCT) of the abdomen is a recently identified aggressive neoplasm. Very few cases have been reported in the literature. Thus, the treatment guidelines are yet to be defined. The role of chemotherapy, radiotherapy and surgery is evolving. We treated four cases of DSRCT involving the abdomen using combination chemotherapy and/or tumor cytoreductive surgery. There were two men and two women. The chemotherapy drugs consisted of cisplatin, adriamycin, etoposide, ifosphamide, vincristine and cyclophsophamide. All patients achieved meaningful partial response to chemotherapy, which maintained for 6-9 months. There were very minimal chemotherapy-related complications. At the time of reporting, the median survival time was 15 months. Thus, DSRCT is an aggressive intra-abdominal tumor with excellent chemoresponsiveness, but relapse is frequent.


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