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Il Giornale di Chirurgia

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

Casistica clinica, 367 - 370
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Abstract
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English version
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.

Versione italiana
I tumori desmoplastici a cellule rotonde sono delle neoplasie molto rare ed aggressive, con peculiari caratteristiche istologiche ed immunoistochimiche, che interessano soprattutto i maschi in giovane età.
Presentiamo un caso di un paziente di 17 anni affetto da tumore desmoplastico a cellule rotonde che si è manifestato con una voluminosa massa in ipocondrio sinistro ed è stato trattato con approccio combinato chirurgico e polichemioterapico.
Ad oggi per tale patologia non vi è trattamento standard e, quando possibile, un approccio chirurgico aggressivo e un trattamento polichemioterapico sembra la soluzione per migliorare sia la sintomatologia che la prognosi.

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