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G Chir. 2007 Oct;28(10):367-70.

Combined resection and multi-agent adjuvant chemotherapy for intra-abdominal desmoplastic small round cell tumour: case report and review of the literature.

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  • 1Azienda Unitá Sanitaria Locale Bologna, Ospedale "SS Salvatore", Bologna.


Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histological and immunohistochemical features occurring in young population with male predominance. We report a case of DRSCT occurred in a 17 years old patient which presented with a large upper left quadrant abdominal mass that was treated with a very aggressive surgical approach and multi-agent chemotherapy. At a 12 months follow-up he is free of recurrence. This kind of tumour has a very poor prognosis. No standard treatment protocol has been established. Aggressive surgery combined with postoperative multi-agent adjuvant chemotherapy is justified not only to relieve symptoms but also to try to improve the outcome.

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