title: Primary Ewing's sarcoma of vulva: a case report and a review of the literature.
abstract: Ewing sarcomas/peripheral primitive neuroectodermal tumors (ES/pPNET) are extremely rare in the vulva. A review of the literature reveals only 14 previously reported possible cases. Here we reported a case of primary extraskeletal Ewing's sarcoma (EES) of the vulva in a 37-year-old woman. Characteristic histologic features of ES/pPNET were present in this case, including a monomorphic population of small round blue cells with cytoplasmic glycogen confirmed by periodic acid-Schiff, membrane staining with CD99 and nuclear staining with FLI-1. After surgery, the patient was found to have pulmonary metastasis and then received six cycles of polychemotherapy. She is still alive with stable disease after 1 year of follow up. Our findings underline the crucial role of immunohistochemical techniques in the differential diagnosis of small round cell tumors in these unusual locations. We also give a summary about the clinical and pathological features of the primary ES/pPNET in the vulva reported previously in the literature.
authors.affiliation: Department of Radiation Oncology, First Affiliated Hospital, Xi'an Jiaotong University School of Medicine, Xi'an, 710061, China. chersm@126.com
authors.forename: Shao-Min
authors.initials: SM
authors.lastname: Che
authors.forename: Pei-Long
authors.initials: PL
authors.lastname: Cao
authors.forename: Hong-Wei
authors.initials: HW
authors.lastname: Chen
authors.forename: Zi
authors.initials: Z
authors.lastname: Liu
authors.forename: Du
authors.initials: D
authors.lastname: Meng
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000473:pathology; D012512:Sarcoma, Ewing / Q000473:pathology*; D014844:Vulva / Q000473:pathology*; D014846:Vulvar Neoplasms / Q000473:pathology*