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Indian Journal of Cancer
Medknow Publications on behalf of Indian Cancer Society
ISSN: 0019-509X
EISSN: 0019-509X
Vol. 42, No. 2, 2005, pp. 78-84
Bioline Code: cn05015
Full paper language: English
Document type: Research Article
Document available free of charge

Indian Journal of Cancer, Vol. 42, No. 2, 2005, pp. 78-84

 en Desmoplastic small round cell tumor: Extra abdominal and abdominal presentations and the results of treatment
Biswas G, Laskar S, Banavali SD, Gujral S, Kurkure PA, Muckaden M, Parikh PM, Nair ChandrikaN

Abstract

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare malignant neoplasm of adolescent males. Current multimodality treatment prolongs life and rarely achieves cure.
Aim : To review the presenting features, histopathology and outcome of 18 patients with DSRCT treated at a single institution.
Setting and Design : This is a retrospective observational study of patients with DSRCT who presented at the Tata Memorial Hospital between January 1994 to January 2005.
Materials and Methods: Eighteen patients of DSRCT seen during this period were evaluated for their clinical presentation, response to chemotherapy and other multimodality treatment and overall survival. The cohort of 18 patients included 11 males (61%) and 7 females (39%) with a mean age of 16 years (Range 1½ - 30 years). Majority (83%) presented with abdomino-pelvic disease. The others, involving chest wall and extremities. There were 6 patients (33%) with metastatic disease at presentation.
Results: The treatment primarily included a multimodality approach using a combination of multiagent chemotherapy with adjuvant surgery and radiotherapy as applicable. A response rate of 39% (CR-1, PR-6), with chemotherapy was observed. The overall response rate after multimodality treatment was 39% (CR-5, PR-2). The overall survival was poor except in patients who had complete excision of the tumor.
Conclusion: Abdomino-pelvic site was the commonest presentation, the disease can occur at other non-serosal surfaces also. Despite aggressive treatment the outcome was poor. However, complete surgical excision seems to provide a better survival.

Keywords
Desmoplastic round cell tumor, Management and outcome

 
© Copyright 2005 Indian Journal of Cancer.
Alternative site location: http://www.indianjcancer.com/

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