Xue-Rui He1,2, Zheng Liu3, Jing Wei1, Wan-Jun Li3, Tao Liu4. 1. Department of Ophthalmology, 3201 Hospital, Xi'an Jiaotong University Health Science Center, 783 Tianhan Ave, Hanzhong, 723000, Shaanxi Province, China. 2. Department of Ophthalmology, Xi'an Hospital, Aviation Industry Corporation of China, Beijing, China. 3. Department of Pathology and Molecular Medicine, 3201 Hospital, Xi'an Jiaotong University Health Science Center, Hanzhong, Shaanxi Province, China. 4. Department of Ophthalmology, 3201 Hospital, Xi'an Jiaotong University Health Science Center, 783 Tianhan Ave, Hanzhong, 723000, Shaanxi Province, China. taoliustone@outlook.com.
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.
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.
Entities:
Keywords:
Desmoplastic small round cell tumor; Fluorescence in situ hybridization; Histology; Immunohistochemistry; Left orbit
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