Key result
A rare case of primary vaginal Ewing sarcoma was confirmed by EWS gene rearrangement, adding to the 7 previously reported cases in this location.
Case Report (n=1)
This case report adds to the limited literature on vaginal Ewing sarcoma, highlighting its typical morphological features and molecular confirmation.
Documents eighth vaginal ES/PNET case; extends sparse literature but leaves optimal management open.
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.
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Bancalari et al. (2011) conducted a case report in Vaginal Ewing sarcoma / primitive neuroectodermal tumor (n=1). A rare case of primary vaginal Ewing sarcoma was confirmed by EWS gene rearrangement, adding to the 7 previously reported cases in this location.
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