title: Ewing's sarcoma/peripheral primitive neuroectodermal tumors of the uterus confirmed with fluorescence in situ hybridization in a 29-year-old Chinese female: a case report and published work review.
abstract: Ewing's sarcoma/peripheral primitive neuroectodermal tumors (ES/pPNET) are a group of small round cell sarcomas that show varying degrees of neuroectodermal differentiation characterized by translocation involving the EWS gene. Uterine ES/pPNET is a rare entity. A 29-year-old Chinese female who presented with abdominal swelling and pain was diagnosed with a primary uterine ES/pPNET on the basis of clinicopathologic, immunohistochemical and fluorescence in situ hybridization (FISH) data. She was given a multimodal treatment, including neoadjuvant, 95% cytoreductive, chemotherapy and radiotherapy. The patient is currently alive with persistent disease after 18 months of follow-up. We emphasized the crucial role of molecular techniques in the differential diagnosis of small round cell tumors in this unusual location. Multimodal therapy may improve the outcomes of patients.
authors.affiliation: Department of Gynecology, West China Second University Hospital of Sichuan University, Chengdu, China.
authors.forename: Tianjin
authors.initials: T
authors.lastname: Yi
authors.forename: Ping
authors.initials: P
authors.lastname: Wang
authors.forename: Lin
authors.initials: L
authors.lastname: Lin
authors.forename: Wei
authors.initials: W
authors.lastname: Jiang
mesh_terms: D000328:Adult; D002148:Calmodulin-Binding Proteins / Q000235:genetics*; D005260:Female; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis* / Q000235:genetics; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics*; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000235:genetics; D014594:Uterine Neoplasms / Q000175:diagnosis* / Q000235:genetics
chemical_list: D002148:Calmodulin-Binding Proteins; C500986:EWSR1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins
keywords: Ewing's sarcoma/peripheral primitive neuroectodermal tumors; fluorescence in situ hybridization; multimodal therapy; uterus