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Search: Organ site Search: Tumor / Syndrome
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WHO Classification of Tumours
Desmoplastic small round cell tumor
Retroperitoneum and peritoneum


Definition

An aggressive malignant neoplasm of uncertain lineage. It is characterized by the presence of small round cells in a desmoplastic stroma. It usually affects children and young adults. The most common site of involvement is the abdomen. Patients usually present with abdominal distention, pain, ascites, and a palpable abdominal mass. The prognosis is usually poor.