title: Primary vaginal Ewing sarcoma: case report and review of the literature.
abstract: Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) are round-cell sarcomas with varying degrees of neuroectodermal differentiation; they are considered members of the same family of tumors based on their common genetic profile. ES/PNET are very infrequent in the female genital tract, the vagina being the rarest gynecological location, with 7 cases reported to date. The authors present a case of vaginal ES/PNET with typical morphological and immunohistochemical features. EWS gene rearrangement was demonstrated by fluorescent in situ hybridization analysis. Of the 7 previously reported cases, only 2 have molecular confirmation. The age at presentation for vaginal ES/PNET is slightly higher than that for these neoplasms in bone and soft tissues, with only 1 reported patient younger than 30 years. The outcome seems to be similar to that of other superficially sited ES/PNET and more favorable than for those observed in the more typical locations. However, the currently available data are limited, and therefore, a larger number of cases is necessary to draw any firm conclusions.
authors: Servicio de Anatomía Patológica, Hospital Universitario de Fuenlabrada, Madrid, Spain. ebancalari.hflr@salud.madrid.org
authors: Eugenia
authors: E
authors: Bancalari
authors: Enrique
authors: E
authors: de Álava
authors: Juan C
authors: JC
authors: Tardío
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D003131:Combined Modality Therapy; D005260:Female; D015321:Gene Rearrangement; D013509:Gynecologic Surgical Procedures; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D034802:RNA-Binding Protein EWS / Q000235:genetics; D012512:Sarcoma, Ewing / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D014625:Vaginal Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy
chemical_list: D014408:Biomarkers, Tumor; D034802:RNA-Binding Protein EWS