Primary Ewing sarcoma of the uterine cervix is an exceptionally rare and aggressive malignancy, with only 34 cases reported in the literature. Early recognition and prompt multimodal treatment are essential due to its poor prognosis. We report a case involving a 32-year-old multiparous woman who presented with foul-smelling vaginal discharge and irregular bleeding. Clinical examination revealed a 6×6 cm friable cervical mass extending into the upper vagina. Imaging demonstrated an FDG-avid cervical mass with regional lymphadenopathy. Histopathological analysis, supported by immunohistochemistry and fluorescence in situ hybridization, confirmed Ewing sarcoma with EWSR1 gene rearrangement. The patient received neoadjuvant chemotherapy with VDC (vincristine, doxorubicin, cyclophosphamide) and PIE (cisplatin, ifosfamide, etoposide) regimens, followed by surgical resection, adjuvant chemotherapy, and pelvic radiotherapy. Although initial disease control was achieved, the patient experienced disease recurrence with distant metastases after a 7-month disease-free interval and succumbed to the illness 9 months after diagnosis. This case underscores the importance of including Ewing sarcoma in the differential diagnosis of rapidly growing cervical tumors, particularly in young women. A multidisciplinary approach involving early molecular diagnostics and aggressive combined therapy is critical to improving clinical outcomes in such rare and aggressive cases.

Extrauterine endometrial stromal sarcoma arising from malignant transformation of the vagina is an extremely rare condition. The diagnosis is often difficult as the symptomatology and pathological features overlap with that of pelvic endometriosis. A 38 years old female presented with complaints of dyspareunia, dysmenorrhea, and painful defecation along with blood-stained vaginal discharge for a year. Examination revealed the presence of multiple brownish irregular nodules in posterior vaginal fornix and fixed tender nodules which on biopsy revealed florid vaginal endometriosis. She improved symptomatically on medical therapy. After 18 months of diagnosis, she presented again with a necrotic growth in posterior fornix, which on repeat biopsy revealed a low-grade endometrial stromal sarcoma. Laparotomy revealed a 7×5 cm mass in the pouch of Douglas, infiltrating the posterior vaginal wall and rectum. A complete cytoreductive surgery with retrograde hysterectomy, excision of posterior vaginal wall and rectosigmoid resection was done. The patient is disease-free at a follow-up of 65 months.