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Search: Organ site Search: Tumor / Syndrome
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WHO Classification of Tumours
Desmoplastic small round cell tumor
Pleura, NOS


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.