The peritoneum is a serious lining of mesothelial cells with a rich vascular and lymphatic capillary network that covers the abdominal and pelvic walls and organs. Peritoneal neoplasm can originate de novo from the peritoneal tissues and defined then as primary peritoneal carcinomatosis (PPC), invade or metastasize into the peritoneum from adjacent or remote organs and will be known as secondary peritoneal carcinomatosis (SPC), No ethnic predisposition or other known risk factors have been identified as specific for the disease. Highly aggressive tumour usually occurs in males during adolescence and early adulthood. Desmoplastic small round cell tumor (DSRCT) is a distinctive clinicopathologic entity and immunophenotypic feature with an aggressive clinical course that typically involves the abdominal and/or pelvic peritoneum of young males. It behaves aggressively and only 29% of patients survive up to 3 years (1).
