Paravaginal peripheral primitive neuroectodermal tumor: A rare tumor

Peripheral primitive neuroectodermal tumor (PNET) of the female genital tract, particularly those in the vaginal and paravaginal region, are extremely rare. A 36-year-old woman presented with clinical features similar to that of a case of cervical fibroid. It was only after surgery that the histopathology suggested it to be a malignant round cell tumour and was CD99 positive. She underwent adjuvant chemotherapy with the Ifosfamide and Etoposide alternating with Vincristine, Doxorubicin, and Cyclophosphamide regime and radical radiotherapy. She is disease free at 12 months of follow-up. The importance of immunostaining and adequate histopathology report lies in the fact that the correct diagnosis thus achieved enabled us to manage a rare case of paravaginal PNET with a multimodality approach.