Isolated Extra-osseous Ewing Sarcoma of Vagina- A Rare Presentation

Primary Ewing Sarcoma of vagina is an extremely rare presentation. The atypical site of occurrence and the lack of ample data predispose it to become a substantial challenge in early diagnosis and management. We report a case of isolated Ewing Sarcoma of vagina in a 31 year old female patient who presented with abnormal vaginal discharge and bleeding for around 2 months and was found to have a vaginal mass on clinical examination. The diagnosis was obtained on the basis of sequential investigations with magnetic resonance imaging (MRI), histopathologic and immunohistochemical (IHC) analysis of the lesion followed by the detection of pathognomonic EWSR1 gene rearrangement by FISH (Fluorescent in situ hybridization). The patient was treated aggressively with multimodal strategy including systemic chemotherapy (VAC/IE protocol), followed by surgical resection of the residual disease and local therapy with radiation. However, she had developed local disease recurrence during her treatment which was further salvaged with a combination of systemic chemotherapy (VIT protocol), En-bloc resection and local radiotherapy followed by maintenance Pazopanib to which she had poor tolerance and the interim scan showed development of multiple hepatic metastases. The patient was transitioned to palliative care and she succumbed to her disease very rapidly.