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INTRODUCTION Pleomorphic leiomyosarcoma is a rare, aggressive soft tissue tumor with poor prognosis. This case study highlights diagnostic challenges, treatment approach, and outcomes in managing pleomorphic leiomyosarcoma to inform clinical practice. AIMS AND OBJECTIVE The aim is to present a comprehensive analysis of a case of pleomorphic leiomyosarcoma, focusing on its clinical presentation, diagnostic process, treatment modalities, and outcomes. Objectives include enhancing understanding of this rare tumor and improving management strategies for similar cases. MATERIAL AND METHODS This single case study examines a 62-year-old female patient presenting with vaginal bleeding and a palpable vaginal mass. Diagnostic imaging, including MRI, was utilized to assess the tumor's extent. A biopsy confirmed pleomorphic leiomyosarcoma. The patient underwent neoadjuvant chemotherapy, followed by radical surgical resection of the tumor. Postoperative clinical evaluations and follow-up appointments monitored for recurrence and complications. Data collected included histopathological findings, imaging results, treatment response, and patient outcomes to inform management strategies for this rare tumor in the vagina. RESULTS The patient underwent successful surgical resection of the pleomorphic leiomyosarcoma, with clear margins achieved. Histopathological analysis confirmed the diagnosis, revealing high mitotic activity and necrosis. During follow-up, imaging showed no signs of local recurrence or metastasis, and the patient reported significant improvement in quality of life. This case illustrates the potential for positive outcomes with early diagnosis and comprehensive treatment of vaginal pleomorphic leiomyosarcoma. CONCLUSION This case of pleomorphic leiomyosarcoma in the vagina underscores the importance of early diagnosis and aggressive treatment. Successful surgical resection with adjuvant chemotherapy, resulted in positive outcomes and no recurrence at one year. This highlights the necessity for heightened awareness of this rare tumor, encouraging prompt evaluation of atypical vaginal symptoms. Effective management strategies can significantly improve prognosis and quality of life for patients with pleomorphic leiomyosarcoma, emphasizing the need for multidisciplinary care.