Desmoplastic small round cell tumour (DSRCT) is a rare malignant tumour which affects mostly young males and has a poor prognosis. Since 1991, when it was first described as a distinct clinical entity by Gerald WL and Rosai J, some 200 cases were reported. DSRCT arises mainly from the abdominal and pelvic cavity, causes abdominal pain or discomfort, weight loss, urinary, bowel or bile obstruction due to compression. Metastases appear most frequently in the liver and lungs. Multimodal therapy is usually indicated with chemotherapy, surgery, radiotherapy, autologous stem cell transplantation. DSRCT has to be differentiated from other small, blue round cell tumours, especially hematopoietic malignancies (leukaemia, lymphoma), neuroblastoma, Ewing sarcoma, PNET, rhabdomyosarcoma, malignant mesothelioma, small cell carcinoma, Wilms tumour. Here we report an extensive abdominopelvic desmoplastic small round cell tumour with liver metastases in an adolescent male patient, highlight the alert deterioration of the clinical status of the patient after the biopsy, the need for second review of the histo-pathological material to obtain the correct diagnosis, the chemoresistance of the tumour besides an apparently good clinical status, and the severe prognosis of this type of tumour.
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