Primary desmoplastic small round cell tumor in the left orbit: a case report and literature review

  • Xue-Rui He
  • Zheng Liu
  • Jing Wei
  • Wan-Jun Li
  • Tao Liu
Case Report



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.


Desmoplastic small round cell tumor Left orbit Histology Immunohistochemistry Fluorescence in situ hybridization 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

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.


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Copyright information

© Springer Science+Business Media B.V., part of Springer Nature 2018

Authors and Affiliations

  • Xue-Rui He
    • 1
    • 2
  • Zheng Liu
    • 3
  • Jing Wei
    • 1
  • Wan-Jun Li
    • 3
  • Tao Liu
    • 1
  1. 1.Department of Ophthalmology, 3201 HospitalXi’an Jiaotong University Health Science CenterHanzhongChina
  2. 2.Department of Ophthalmology, Xi’an HospitalAviation Industry Corporation of ChinaBeijingChina
  3. 3.Department of Pathology and Molecular Medicine, 3201 HospitalXi’an Jiaotong University Health Science CenterHanzhongChina

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