Abstract: Background and purpose: Primary female genital system lymphoma (PFGSL) is a rare malignant tumor of female genital system. Its incidence rate is extremely low, and most reports are case reports. This paper aimed to analyze clinical features, diagnosis, treatment and prognosis of PFGSL. Methods: A retrospective analysis of clinical data of 57 PFGSL patients, treated in Fudan University Shanghai Cancer Center from Jan. 2008 to Dec. 2019 was conducted. All the patients met the PFGSL diagnostic criteria. Patients were divided into two groups: single treatment group (33cases) and comprehensive treatment group (24 cases). The median follow-up time was 37.5 months (2.0-85.0 months). Forty out of 57 patients’ local lesions were located in cervix (70%), 13 in ovary (22.8%), 3 in uterus (5.3%) and 1 in vagina (1.8%). According to the International Prognostic Index (IPI) score, 36 patients were low-risk (63.2%) and 21 patients were middle-risk (36.8%). As to lymphoma stage, 36 cases were in stage ⅠE (63.2%), 19 cases were in stage ⅡE (33.83%), and 2 cases were in stage ⅢE (3.5%). Out of 57 patients, the pathologic type of 42 patients were diffuse large B-cell lymphoma (73.7%). Results: Fourteen cases had recurrence, in which twelve were from single treatment group and two from the comprehensive treatment group. The recurrence rate was 24.6% (14/57). Four cases recurred in central nervous system, 1 in eyeball, 4 had loco-regional recurrence and 5 had systemic metastases. The 3-year and 5-year overall survival (OS) rates of 57 patients were 82% and 78%, respectively. The 3-year and 5-year disease-free survival (DFS) rates of 43 patients were 77% and 71%, respectively. The recurrence rate of single treatment group was 36.4% (12/33). In single treatment group, the 3-year and 5-year OS rates were 72% and 64% respectively; the 3-year and 5-year DFS rates were 67% and 57% respectively. In the comprehensive treatment group, the 3- and 5-year OS rate was 95%, and the 3- and 5-year DFS rate was 91%. Multivariate analysis showed that the OS and DFS rates were all significantly correlated with treatment (P<0.05). Conclusion: PFGSL has very low incidence rate and nonspecific clinical manifestations. It is easily misdiagnosed. Gynecological doctors should improve the understanding of this disease and work with chemoradiotherapy doctors to treat this disease.

Key words: Primary female genital system lymphoma, Comprehensive treatment, Prognosis