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Extrauterine endometrial stromal sarcoma: A systematic review and outcome analysis
Affiliation:1. Department of Pathology, Ankara Training and Research Hospital, Ankara, Turkey;2. Department of Biomedical Engineering, TOBB University of Economics and Technology, Ankara, Turkey;3. Department of Medical Microbiology, Gulhane Institute of Health Sciences, Ankara, Turkey;4. Department of General Surgery, Gülhane Education and Research Hospital, Ankara, Turkey;1. Chulabhorn International College of Medicine, Thammasat University, Pathumthani, Thailand;2. Division of Pathology, Thammasat University Hospital, Pathumthani, Thailand;3. Department of Pathology, King Chulalongkorn Memorial Hospital, Bangkok, Thailand;1. Department of Cardio-Thoracic Surgery, Zhoushan Hospital, 739 Dingshen Road, Zhoushan, Zhejiang 316000, PR China;2. Department of Pathology, Zhoushan Hospital, 739 Dingshen Road, Zhoushan, Zhejiang 316000, PR China;1. Evidence-Based Practice Center, Mayo Clinic, Rochester, USA;2. Department of Laboratory Medicine and pathology, Mayo Clinic, Rochester, USA
Abstract:Endometrial stromal sarcoma (ESS) is the second most common uterine mesenchymal neoplasm. ESS can arise from extrauterine locations without any uterine involvement and is called extrauterine ESS (EESS). The epidemiological features of EESS are not well-known. Moreover, the factors affecting its outcome have not been systemically studied. The treatment of EESS closely follows that of uterine ESS, comprised of different combinations of surgical management, hormone therapy, chemotherapy, and radiation therapy. However, the effectiveness of different treatment protocols for EESS has not been studied. Here, we have performed a systematic review of all reported cases of EESS in the English literature. We further performed a meta-analysis of the outcome data and investigated how the patients' age, tumor site, tumor size, and management affect the overall and progression-free survival of the patients. We found that tumor site and mode of treatment significantly affected the overall survival and progression-free survival of the patients. Tumor size significantly affected overall survival but not progression-free survival, while the age at diagnosis did not affect patient outcome. As far as we know, ours is the first systematic study of this rare malignancy with an emphasis on outcome analysis.
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