desmoplastic small round cell tumour
Pronunciation: dez-moh-PLAS-tik smal rownd sel TOO-mer
A rare, highly aggressive soft-tissue sarcoma predominantly affecting adolescents and young adults, characterised by nests of small round cells embedded in a dense desmoplastic stroma and a pathognomonic EWSR1–WT1 gene fusion.
Full Definition
Desmoplastic small round cell tumour (DSRCT) most commonly arises in the peritoneum of adolescent males but can involve any serosal surface. The hallmark molecular finding is the t(11;22)(p13;q12) translocation producing an EWSR1–WT1 fusion transcript, which also serves as a diagnostic marker. Histologically, the tumour shows polyphenotypic differentiation, co-expressing epithelial, mesenchymal, and neural markers. Prognosis is poor despite multimodal therapy. Editors must hyphenate 'desmoplastic small round cell tumour' as a compound modifier before a noun (e.g., 'desmoplastic-small-round-cell-tumour cells' is incorrect; the full phrase should not be over-hyphenated). DSRCT is the standard abbreviation.
Usage
Usage note: Abbreviate as DSRCT after first full use. Do not conflate with other small round blue cell tumours of childhood.
In Context
- "FISH analysis confirmed the EWSR1–WT1 fusion, establishing the diagnosis of desmoplastic small round cell tumour." — Molecular pathology report
- "DSRCT should not be listed under 'Ewing family tumours' in the manuscript, as it is a distinct entity." — Manuscript peer-review annotation