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Primary Ewing's sarcoma-primitive neuroectodermal tumor of the vagina
Xiaoyun Liao1, Xiuxia Xin, Xiaohong Lü
1Department of Pathology, Peking University People's Hospital, Beijing 100044, PR China. xiaoyliao@sina.com
Background:
Only three cases of Ewing's sarcoma-primitive neuroectodermal tumor (ES-PNET) occurring in the vagina have been previously reported.
Case:
A 30-year-old Chinese woman presented with a vaginal mass measuring 5 cm in greatest dimension. The tumor was composed of solid sheets of undifferentiated small round cells with numerous Homer-Wright rosettes. Immunohistochemically, it was positive for CD99, FlI-1 protein, synaptophysin, neuron-specific enolase, vimentin, and S-100 protein. The patient underwent wide local excision of the tumor and subsequent total abdominal hysterectomy with postoperative chemotherapy and radiotherapy. She is disease-free 36 months after the initial surgery.
Conclusion:
This is a rare case of ES-PNET occurring in the vagina and the first showing many distinctive Homer-Wright rosettes. A review of literature indicates that vaginal or vulvar ES-PNET affects younger women, and it may have a relatively favorable outcome.