Primary desmoplastic small round cell tumour of the prostate.

Desmoplastic small round cell tumour (DSRCT) is a highly aggressive soft-tissue sarcoma with distinctive morphological features and characteristic EWSR1::WT1 gene fusion. DSRCT occurs in a variety of anatomic sites, with abdominal cavity being the most common location. Primary DSRCTs arising in the male genital system are exceedingly rare, with no documented definitive cases of primary DSRCT of the prostate to date, although 28 cases of DSRCT in the testicular or paratesticular regions have been reported. We here present two cases of primary DSRCT of the prostate. Both cases demonstrated the distinct morphology and the typical multiphenotypic immunohistochemical profile, and the characteristic EWSR1::WT1 fusion verified by fluorescent in situ hybridisation. Our cases expand the anatomic distribution of primary DSRCT and highlight the importance of considering this rare tumour in the differential diagnoses of small cell malignancies of the prostate. © Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group.

Citation

Jingyu Qian, Yanjin Yang, Xin Xie, Yifan Kang, Jinjing Zhong, Xueqin Chen, Ni Chen, Qiao Zhou, Ling Nie. Primary desmoplastic small round cell tumour of the prostate. Journal of clinical pathology. 2024 Dec 18;78(1):64-69

Mesh Tags

Substances

PMID: 39074976

search → result