Primary desmoplastic small round cell tumor in the left orbit: a case report and literature review
- 72 Downloads
Desmoplastic small round cell tumor is a rare malignant neoplasm that most often occurs in the abdomen or pelvis of young men. We herein describe a rare case of desmoplastic small round cell tumor arising from the left orbit in a 16-year-old male.
Methods and results
A biopsy was performed and the histology showed the nests of tumor cells with small round cell morphology. The tumor cells showed immunopositivity for desmin, CD99, CD56, SMA, NSE, CgA, SYN, Ki67 and vimentin. Fluorescence in situ hybridization study using EWSR1 break-apart probe was positive for EWSR1 gene rearrangement. After complete surgical resection of the tumor, we did not find tumor recurrence or metastasis with one-year follow-up. Furthermore, a review of the relevant English literature has been discussed.
In the present study, for the first time, we report a case of desmoplastic small round cell tumor which is located in the orbital region.
KeywordsDesmoplastic small round cell tumor Left orbit Histology Immunohistochemistry Fluorescence in situ hybridization
This study was funded by the Key Research and Development Program of Shaanxi Province (2017ZDXM-SF-067).
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.
- 12.Wang L, Bhargava R, Zheng T, Wexler L, Collins MH, Roulston D, Ladanyi M (2007) Undifferentiated small round cell sarcomas with rare EWS gene fusions: identification of a novel EWS-SP3 fusion and of additional cases with the EWS-ETV1 and EWS-FEV fusions. J Mol Diagn 9:498–509CrossRefPubMedPubMedCentralGoogle Scholar