Objective:

To present the case of a female patient diagnosed with cervico-vaginal extraosseous Ewing's sarcoma and to conduct a review of the literature, with special emphasis on histopathological diagnosis and therapeutic options for these neoplastic lesions.

Materials and methods:

Woman of a 53 year-old on follow-up due to a history of infiltrating ductal breast cancer, who presents with abnormal genital bleeding. On physical examination, a cervico-vaginal mass extending to the pelvis was observed. A biopsy was taken and the result was consistent with Ewing's sarcoma/primitive neuroectodermal tumour of the cervix. A search was conducted in the Medeline databases via PubMed, using the terms "sarcoma; ewing", "neuroectodermal tumours", "primitive", "peripheral" and "uterine cervical neoplasms", looking for reviews of the literature, case reports and clinical case series both in English and Spanish, published since 2009.

Results:

Overall, 12 articles were found, including reviews of the literature, case reports and clinical case series, describing the most relevant aspects of the treatment, clinical manifestations and molecular, immunohistochemical and histopathological diagnosis of this disease.

Conclusions:

Gynaecological extraosseous Ewing's tumour is an extremely rare lesion whose diagnosis is based on the study of the histopathological morphology, immunohistochemical tests and genetic testing. A combination of surgery, chemotherapy and radiotherapy is considered the treatment of choice at the present time.

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