Intra-abdominal desmoplastic small round cell tumour

Intra-abdominal desmoplastic small round cell tumour is a rare tumour which has only recently been described. It demonstrates epithelial, mesenchymal and neural features and has an aggressive clinical course. We report a case of a 15 year old male with this tumour who, after debulking surgery and chemotherapy, relapsed with disease 1 month later. After further debulking surgery, extensive chemotherapy and bone marrow transplantation he relapsed with disease 5 months later and died. Total survival was 20 months.