Intraabdominal desmoplastic small round cell tumour

Abstract

Desmoplastic small round cell tumour (DSRCT) is an extremely rare neoplasm. Adolescent males and young adults are most frequently affected. It is highly malignant, with only 29% of patients surviving up to 3 years. This paper documents two cases, one of which, at 4 years old, is the second youngest case documented. Case 1, a 10-year old boy, presented with a 20-day history of choluria, acholia, asthenia, anorexia, and right abdominal pain. Laboratory values were altered, and imaging showed multiples masses in the liver and retroperitoneum. A minilaparotomy was carried out, and a biopsy showed a stage III DSRCT. He was treated with chemotherapy but died of hepatic failure. Case 2, a 4-year-old boy, presented with a 2-month history of abdominal distension. Several hard masses were palpated in the abdomen, and a right inguinal mass that compressed the right testis was observed. Biopsy of the inguinal tumour showed a DSRCT. After treatment with chemotherapy, two operations were carried out to resect different intraabdominal masses. The patient died with peritoneal carcinomatosis 2 months after the last operation. The first patient died due to the advanced stage of the disease, and the second died after chemotherapy, peripheral blood stem transplantation, and multiple operations. The occurrence of this type of tumour in the paediatric age group as well as its high malignancy is noteworthy. Until more effective forms of treatment are found, we recommend treatment with chemotherapy, surgery, and radiotherapy, with close monitoring of the patient.