Moon Joo  Kim,Renee K   Eng, Ahmed N Shehabeldin,Yimin  Ge,Mary R Schwartz,Yun  Wu,Jae Y Ro,
Desmoplastic Small Round Cell Tumor with an Unusual Site and Age Presentation: A Case Report and Literature Review
Surgical Case Reports
2020
2613-5965
http://dx.doi.org/10.31487/j.SCR.2020.08.11
https://www.sciencerepository.org/desmoplastic-small-round-cell-tumor-with-an-unusual-site_SCR-2020-8-111 
Abstract: Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive sarcoma usually arising in the abdomen
or pelvis of young males under 30 years of age. We present a case of a 32-year-old male with a palpable
axillary mass and multiple bilateral lung nodules. Excisional biopsy of the axillary mass showed sheets and
nests of small round cells with numerous mitoses, areas of necrosis, and desmoplastic stroma. Initial
immunohistochemical stains performed at an outside institution demonstrated immunoreactivity for
epithelial markers and focal reactivity for breast markers. The tumor was initially diagnosed as poorly
differentiated carcinoma, with consideration of a possible primary breast cancer. Additional workup
demonstrated strong, diffuse positivity for desmin in tumor cells, leading to the final diagnosis of DSRCT.
Subsequent molecular testing confirmed DSRCT with EWSR1 gene rearrangement. This case illustrates the
importance of recognizing the morphologic features of DSRCT in the setting of uncommon location or
patient age.Keywords: Carcinoma, desmin, desmoplasia, EWSR1 gene rearrangement, small round cell tumor