Abstract
Desmoplastic small round cell tumor (DSRCT) is a somewhat chemotherapy-sensitive, but highly lethal, sarcoma diagnosis. Distribution by age for adults is shown in Fig. 19.1, emphasizing that it is an uncommon tumor, seen mainly in adolescents and young adults (age 15–30). There is a strong male predominance (∼5:1) and nearly always affects the peritoneum as multifocal/metastatic disease (Fig. 19.2). The best outcomes from therapy are those who have both a good response to chemotherapy (using agents typically employed for Ewing sarcoma) and successful surgical debulking [1]. Rare patients will present with disease elsewhere; if disease is localized, the cure rate is expected to be higher than the 5–15 % typically encountered for those patients with primary abdominal disease [1, 2].
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Brennan, M.F., Antonescu, C.R., Maki, R.G. (2013). Desmoplastic Small Round Cell Tumor. In: Management of Soft Tissue Sarcoma. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5004-7_19
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