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Category: Volume 10 Issue 07 July 2022
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Title: Desmoplastic Small Round Cell Tumor of Small Intestine: A Case Report and Review of Literature

Authors: Pawan Kumar, Diptajit Paul, Abhishek Soni, Vivek Kaushal

 DOI: https://dx.doi.org/10.18535/jmscr/v10i7.06

Abstract

Desmoplastic small round cell tumor (DSRCT) is an uncommon entity characterized by sarcomatous abdomino-pelvic mass arising from serosal surface of peritoneum. Till date, only a few cases of DSRCT has been reported in literature. Here we presented a case of small intestinal DSRCT in a young male presented with peri-umbilical pain of 3-months duration. Patient underwent exploratory laparotomy with resection of large ileal mass. Histopathology of surgical specimen revealed desmoplastic small round cell tumor.  Patient was planned for adjuvant chemotherapy with VAC regimen. But after receiving 2-courses, patient condition deteriorated and patient was succumbed to death. This case report demonstrated the aggressiveness of DSRCT. 

Keywords: Adjuvant chemotherapy, desmoplastic small round cell tumor, exploratory laparotomy, VAC regimen.

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Corresponding Author

Dr Diptajit Paul

Pt BD Sharma PGIMS, Rohtak, Haryana India 124001