Desmoplastic Small Round Cell Tumor-Reprot of 3 Cases And Review of the Literature

Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm with distinctive histological features, characterized by extensive stromal tissue around islands of small and undifferentiated cells revealing the desmoplastic appearance. It was first described by Gerald and Rosai. It mainly the peritoneum and is found predominently in adolescents and young adults, and it is much more common in males than in females. The histological and immunhistochemical features are very distinct but may be misdiagnosed as other undifferentiated neoplasm if the pathologist has no idea of this entitiy. We report the experience of three cases here. The first case was diagnosed as malignant neuroendocrine neoplasm orginally and revised to DSRCT later. Case 2 had been diagnosed as Atypical carcinoid and received surgery and chemotherapy. It was revised as DSRCT 5 years later by an other pathologist. Case 3 carried the characteristic feature whereby diagnosis could be made even in frozen section.