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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2010; 31(01): 24-27
DOI: 10.4103/0971-5851.68849
CASE REPORT

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

Biswa Mohan Biswal
Department of Nuclear Medicine, Radiotherapy and Oncology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kelantan, Malaysia
,
Venkatesh R Naik
Department of Pathology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kelantan, Malaysia
,
Syed Ejaz Shamim
Department of Nuclear Medicine, Radiotherapy and Oncology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kelantan, Malaysia
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Abstract

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.

Keywords

Abdomen - chemotherapy - desmoplastic small round cell tumor - management and prognosis - radiotherapy - surgery


Publication History

Article published online:
19 November 2021

© 2010. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

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