A Case of Desmoplastic Small Round Cell Tumor / 대한소아혈액종양학회지

Desmoplastic small round cell tumor (DSRCT) is a distinct clinicopathologic entity that has only recently been described. This aggressive malignant small cell neoplasm tends to occur in adolescents and young adults, who may present with vague abdominal discomfort or distention. We report a case of DSRCT in a 13 year-old girl who presented with intermittent abdominal pain and bulky intra-abdominal mass, accompanied by an increased serum level of a tumor marker CA125. Clinical, pathological, immunohistochemical and radiologic features were compatible with DSRCT. She is under aggressive combination chemotherapy after surgical resection.