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Desmoplastic small round cell tumors are  mesenchymal tumors affecting young adults which are rare and poorly understood. Due to its rarity there are no standard protocols for staging and management of these tumors. We report a case of a 26 year old male, presenting with an  abdominal mass with no other associated symptoms. He underwent a debulking surgery following ultrasound guided biopsy which was diagnostic of desmoplastic small round cell tumor. Immunohistochemistry is vital for diagnosis of these tumors. Early diagnosis and multimodal treatment with radical surgery, chemotherapy and radiotherapy are essential in management of this rapidly progressive malignancy, however prognosis is poor.


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