Desmoplastic small round cell tumour presenting as cervical lymph nodes metastases with solid pattern morphology and novel EWSR1-WT1 fusion transcript

Desmoplastic small round cell tumour (DSRCT) is an aggressive, paediatric mesenchymal neoplasm that predominantly involves the abdominal or pelvic peritoneum and often presents with widespread abdominal/peritoneal implants or metastases. Histologically, DSRCT has a characteristic appearance of nests of fairly uniform small round cells setting in variable amounts of desmoplastic stroma and immunohistochemically it exhibits a polyphenotypic multi-directional differentiation, with co-expression of epithelial, neural, and muscle markers.