Importance of studying primitive neuroectodermal tumors and extraosseous Ewings sarcoma of the vagina and vulva

Primitive neuroectodermal tumor (PNT) and Ewing's sarcoma are rare, round-cell tumors, characterized by the presence of the t(11; 22)(q24; q12) chromosomal translocation. A review of the literature revealed only 38 previously reported cases of vulvar PNT and Ewing's sarcoma and 15 vaginal PNT and Ewing's sarcoma. Although rare, these types of tumors should be taken into consideration when making a differential diagnosis for vulvar or vaginal tumors. The currently available data is limited, and therefore, case reports are essential for improving knowledge and management of these types of extremely rare tumors. However, further molecular and histopathological studies are essential for an improved understanding of these conditions and for an early, correct diagnosis. Although the gathered and presented data from the present review are limited, the literature demonstrates that the outcome of these types of cancer are more favorable compared with outcomes observed for carcinomas in more typical locations.