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Desmoplastic Small Round Cell Tumor: Intro & Gross

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INTRODUCTION: Desmoplastic small round cell tumor (DSRCT) is a highly aggressive uncommon tumor that usually involves abdominal or pelvic peritoneum. It consists of undifferentiated small round cells in a background of hyalinized stroma. The tumor cells express epithelial, mesenchymal, and neural markers.

CLINICAL: DSRCT is a tumor of adolescent and young adults with a strong male predominance (M:F = 4:1). The mean age at diagnosis is approximately 22 years with a range from 6 to 49 years. Rare cases occur in the very young or very old individuals. Patients usually present with abdominal distension, pain, and constipation. There may be intestinal obstruction, ureteral obstruction, ascites, and impotence.

GROSS: The tumor is usually multinodular with extensive involvement of abdominal and/or pelvic peritoneum. Rare cases involve paratesticular region, ovary, pleura, CNS, salivary glands, bone, lungs, and kidney etc. Grossly, it has a firm, gray-white cut surface. Areas of hemorrhage, necrosis and cystic change are frequently seen.

Image courtesy of Dr. Jean-Christophe Fournet, Paris, France; humpath.com; Used with permission

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