Intraabdominal desmoplastic small round cell tumour: report of two cases in paediatric patients

Intraabdominal Desmoplastic Small Round Cell Tumour (IDSRCT) is a very rare neoplasia with a unique immunoprofile. Children and young adults are most commonly affected. We report two cases with IDSRCT in children who initially presented with ascites, pain and abdominal mass. Complete surgical excision was possible only in one patient. Although both patients underwent multiagent chemotherapy, they had a relapse of the disease. One patient died two years after diagnosis. The tumour has a very poor prognosis. Survival is correlated to the radical resection of the tumour combined with intense chemotherapy and radiotherapy.