Abstract: Objective To investigate the clinicopathology features of primary female genital system lymphoma (PFGSL) and analysis the diagnosis, treatment and the factors affecting prognosis.Methods Clinical data of 8 PFGSL patients were collected retrospectively and followed up, who were preliminary diagnosed in Beijing Obstetrics and Gynecology Hospital, Capital Medical University from January 2010 to December 2020. Results The mean age of 8 patients at presentation was (54.3±12.6) years. The lesions of PFGSL mainly involved ovary (4 patients), uterus (2 patients), cervix (1 patient), and vulva (1patient). The main clinical manifestations were postmenopausal vaginal bleeding, abdominal distension, ascites and pelvic mass. All the pathological types were B-cell non-Hodgkin's lymphoma (NHL), positive CD20 and high Ki67 in immunohistochemistry. There were 4 patients with stage IIEA, 1 patient with stage IVA and 1 patient with stage IVB. 6 patients were diagnosed with postoperative pathology, and 2 patients were diagnosed with tumor biopsy. Chemotherapy with C/EBP-homologous protein (CHOP) regimen was used in 5 patients. Among them, 1 patient underwent autologous stem cell transplantation,1 patient lost to follow-up, 2 patients death, 5 patients survival (4 patients with tumor-free survival). Conclusion PFGSL is a rare kind of extranodal lymphoma, with no specific clinical manifestation. It is difficult to distinguish from primary gynecological malignancy. The clinician should be vigilant in diagnosing PFGSL when the mass is milky and carcinoma cells can not be found in ascites extraction after repeated careful examination. Further, biopsy or surgical resection of the tumor is required to make a definite pathological diagnosis. The chemotherapy regimen based on CHOP is the main treatment.

Key words: primary female genital system lymphoma, non-Hodgkin's lymphoma;, clinicopathology features