DOI: https://doi.org/10.36347/sjmcr.2025.v13i09.031

Pages: 2074-2081

Primary vaginal sarcomas are rare gynecologic tumors of unknown etiology, characterized by aggressive behavior, a high risk of recurrence, and a poor prognosis. Undifferentiated vaginal sarcoma is an exceptionally uncommon subtype, with no prospective trials or standardized guidelines specifically addressing its management. Surgery is considered the mainstay of therapy for localized and recurrent disease. However, for locally advanced tumors or when surgery would result in excessive morbidity, radiotherapy is considered the standard alternative. We report the case of a 19-year-old woman diagnosed with high-grade undifferentiated vaginal sarcoma, who showed no response to six cycles of VAC chemotherapy and was subsequently treated with exclusive 3D conformal radiotherapy to a total dose of 70 Gy with concomitant chemotherapy, achieving significant clinical improvement. This case illustrates the therapeutic challenges of such rare tumors and highlights the need for individualized management and systematic reporting to improve knowledge and outcomes.