Primary vaginal ewing’s sarcoma/primitive neuroectodermal tumour : diagnostic and treatment challenges

Extra osseous Ewing’s sarcoma/primitive neuroectodermal tumour (PNET) of the genital tract of women is scarcely described in the literature and involvement of the vagina is even rarer with a very few cases reported so far. We present 50-year-old-woman who presented with a vaginal mass that was diagnosed to be a malignant round cell tumour which later was confirmed to be primary vaginal Ewing’s sarcoma/ PNET on light microscopy and immunohistochemical staining. She was then treated with induction chemotherapy followed by local radiotherapy and further maintenance chemotherapy. This rare case of primary vaginal Ewing’s sarcoma/PNET emphasizes the need for combining morphological features with immunohistochemistry with a panel of antibodies in establishing the diagnosis of Ewing’s sarcoma/PNET at an uncommon site. Further, the case also highlights the use of induction chemotherapy followed by radiation therapy and subsequent maintenance chemotherapy as a treatment modality.