CIC‐associated round cell sarcoma of the small bowel

Small round cell sarcoma (SRCS) is considered a highly aggressive soft tissue tumour. The World Health Organization (WHO) has recognized a distinct subtype in view of the recently discovered CIC fusion gene. It has shown to have distinct pathophysiology and prognosis unlike Ewing sarcoma (ES) and rhabdomyosarcoma. However, due to its rarity, no standardized treatment has been concluded. We reported a case of intestinal obstruction caused by small bowel associated associated, laparotomy and small bowel resection was performed with good recovery and 1 year free disease survival. The gold standard of diagnosis with molecular confirmation is utmostly important in order to learn more about the natural history of this subtype thus formulating the best management plan.