Abstract
Purpose
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.
Conclusions
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.
Similar content being viewed by others
References
Leuschner I, Radig K, Harms D (1996) Desmoplastic small round cell tumor. Semin Diagn Pathol 13:204–212
Cao Y, Chen Y, Yang LI, Qian ZH, Han SG, Li QH, Yu RS (2015) Desmoplastic small round cell tumor: a case report of a rare differential diagnosis of solid tumors of the pleura. Oncol Lett 10:2991–2995
Gerald WL, Miller HK, Battifora H, Miettinen M, Silva EG, Rosai J (1991) Intra-abdominal desmoplastic small round-cell tumor. Report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol 15:499–513
Ud Din N, Pekmezci M, Javed G, Horvai AE, Ahmad Z, Faheem M, Navarro AL, López-Terrada D, Perry A (2015) Low-grade small round cell tumor of the cauda equina with EWSR1-WT1 fusion and indolent clinical course. Hum Pathol 46:153–158
Shen XZ, Zhao JG, Wu JJ, Liu F (2014) Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor. World J Gastroenterol 20:5157–5164
Xie YP, Shen YM (2016) Ovarian involvement of a desmoplastic small round cell tumor of unknown primary origin with lymph node and lung metastases: a case report. Oncol Lett 11:1125–1129
Bismar TA, Basturk O, Gerald WL, Schwarz K, Adsay NV (2004) Desmoplastic small cell tumor in the pancreas. Am J Surg Pathol 28:808–812
Gil A, Gomez Portilla A, Brun EA, Sugarbaker PH (2004) Clinical perspective on desmoplastic small round-cell tumor. Oncology 67:231–242
Zhang WD, Li CX, Liu QY, Hu YY, Cao Y, Huang JH (2011) CT, MRI, and FDG-PET/CT imaging findings of abdominopelvic desmoplastic small round cell tumors: correlation with histopathologic findings. Eur J Radiol 80:269–273
Ordóñez NG (1998) Desmoplastic small round cell tumor: II—an ultrastructural and immunohistochemical study with emphasis on new immunohistochemical markers. Am J Surg Pathol 22:1314–1327
Ordóñez NG (1998) Desmoplastic small round cell tumor: I—a histopathologic study of 39 cases with emphasis on unusual histological patterns. Am J Surg Pathol 22:1303–1313
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 9:498–509
Lae ME, Roche PC, Jin L, Lloyd RV, Nascimento AG (2002) Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study of 32 tumors. Am J Surg Pathol 26:823–835
Kushner BH, LaQuaglia MP, Wollner N, Meyers PA, Lindsley KL, Ghavimi F, Merchant TE, Boulad F, Cheung NK et al (1996) Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol 14:1526–1531
Quaglia MP, Brennan MF (2000) The clinical approach to desmoplastic small round cell tumor. Surg Oncol 9:77–81
Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP (2005) Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg 40:251–255
Zhang J, Xu H, Ren F, Yang Y, Chen B, Zhang F (2014) Analysis of clinicopathological features and prognostic factors of desmoplastic small round cell tumor. Pathol Oncol Res 20:161–168
Zhang S, Zhang Y, Yu YH, Li J (2016) Complete response of giant desmoplastic small round cell tumor treated with chemoradiotherapy: a case report. Oncol Lett 11:1069–1072
Ikeue T, Ohi I, Noguchi S, Fukao A, Terashita S, Horikawa S, Sugita T (2016) Desmoplastic small round cell tumor of the pleura successfully treated with a lower dose of pazopanib. Intern Med 55:2463–2467
Funding
This study was funded by the Key Research and Development Program of Shaanxi Province (2017ZDXM-SF-067).
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
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.
Rights and permissions
About this article
Cite this article
He, XR., Liu, Z., Wei, J. et al. Primary desmoplastic small round cell tumor in the left orbit: a case report and literature review. Int Ophthalmol 39, 471–475 (2019). https://doi.org/10.1007/s10792-018-0829-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10792-018-0829-y