Desmoplastic small round cell tumor of the pleura

Last revised by Dr Sally Ayesa on 30 Apr 2022

Desmoplastic small round cell tumor (DSRCT) of the pleura is a rare primary pleural malignancy comprising of mesenchymal cells.

Epidemiology

Tend to occur in younger patients (mean ~ 25.5 years) and with a slightly greater male predilection. 

Treatment and prognosis

DSRCT tend to be aggressive tumors with a poor prognosis.

History and etymology

DSRCT was first described in 1989 by Gerald and Rosai and Ordonez et al. as a highly aggressive and rare mesenchymal tumor arising from serosal surfaces.

See also

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