What is pleural desmoplastic small round cell tumor (DSRCT)?

Updated: Dec 25, 2019
  • Author: Joseph F Tomashefski, Jr, MD; more...
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Answer

Answer

DSRCT was first described by Gerald and Rosai in 1989 as a primitive neoplasm of children and young adults that most frequently occurs in the serosa of the pelvic cavity. [12] Rare cases of primary pulmonary or pleural DSRCT have subsequently been identified. [13, 14]

DSRCT is composed of small cells with round hyperchromatic nuclei and a dense fibrous or spindle cell stroma. [3, 8] The histogenesis of DSRCT is speculated to be “mesothelioblastic,” arising from the submesothelial progenitor cell; however, this concept is controversial and not accepted by all investigators. [15]


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