Ewing sarcoma (ES) represents approximately ten percent of primary malignant bone tumours and one percent of soft tissue tumours. It tends to arise in the diaphysis or metaphyseal-diaphyseal portion of long bones, although any bone may be involved.
Frequently, the first symptoms are pain and swelling.
Twenty percent of ES at diagnosis have radiographic evidence of metastasis. Lungs and bone are the main metastatic locations.
Radiologically, an aggressive osteolytic lesion is commonly observed.
ES is characterized by a morphologically uniform round cell proliferation with round nuclei containing fine chromatin.
CD99 is expressed in nearly all ES and thereby is a highly sensitive immunohistochemical marker.
Several studies have confirmed a characteristic 11, 22 (q24, q12) chromosomal translocation in 85 percent of the cases ; the translocation t (21, 22) and three even rarer translocations (t (7, 22), t(2, 22) t(17, 22)) have also been found.
Necrosis has a strong prognostic value . High dose chemotherapy is used in poor responders .
We found 17 cases of primary ES involving the vagina and/or vulva in the literature. A few cases of primary neuro ectodermal tumors (PNET) in the pelvis have also been reported .
Unusual metastasic locations have been described, for example, the breast , myocardial muscle , paranasal sinuses , iris ), and pancreas . That explains why, even if a second primary cannot be completely excluded in our case, the probability of a metastasis is much higher. To our knowledge, this is the first case ever reported of a vaginal Ewing metastasis.