Desmoplastic small round cell tumour (DSRCT): A rare entity that highlights the importance of immunohistochemistry

Abstract

Desmoplastic small round cell tumour (DSRCT) is a rare malignant tumour of mesenchymal origin1 with a strong male predominance; the mean age at diagnosis is 22 years.2 We present a case of a 31-year-old female with a pelvic mass and widespread lymphadenopathy at laparotomy. Frozen section of mass and pelvic node were performed and reported as a malignant small round blue cell tumour. Paraffin sections showed variably sized nests of small, monotonous, hyperchromatic cells with indistinct borders, numerous mitoses, and prominent desmoplastic stroma.