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Desmoplastic small round cell tumor (DSRCT) is a unique, highly
aggressive neoplasm with characteristic topographic, histologic,
immunohistochemical, cytogenetic and molecular features. It chiefly
affects adolescent males and young adults and is most frequently
presented as a large abdominal mass with widespread peritoneal
involvement at the time of diagnosis. Histologically, it is
composed of nests of small, undifferentiated round or oval
hyperchromatic cells embedded in abundant desmoplastic stroma. A
multidisciplinary treatment including chemotherapy, aggressive
debulking surgery, radiation and myeloablative chemotherapy with
stem cell rescue might be the proper approach to treat this rare
malignancy and may improve progression-free survival. This book
presents current research in the study of the desmoplastic small
round cell tumor.
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