Abstract
Desmoplastic small round cell tumor are uncommon tumor. They occur in young adult and have a strong male predilection. Their localizations are multiple with predominant abdominal pain. They are characterized at histology by undifferentiated small cells in dense desmoplastic stroma. Immunohistochemical stains and specific cytogenetic and molecular characteristics provide the diagnosis. The prognosis of this tumor is poor although the chemotherapy is aggressive.
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