Abstract
Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor, 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 and autologous stem cell transplantation. DSRCT should be differentiated from other small, blue round cell tumors, especially hematopoietic malignancies (leukemia, lymphoma), neuroblastoma, Ewing sarcoma, PNET, rhabdomyosarcoma, malignant mesothelioma, small cell carcinoma and Wilms tumors. We report the case of a patient with an extensive abdominopelvic desmoplastic small round cell tumor, with liver metastases, in an adolescent male patient, highlighting the alert deterioration of the clinical status of the patient after the biopsy, the need for a second review of the histopathological material in order to obtain a correct diagnosis, the chemoresistance of the tumor despite an apparently good clinical status, and the severe prognosis of this type of tumor.