57例原发女性生殖系统淋巴瘤临床特征分析

背景与目的：原发女性生殖系统非霍奇金淋巴瘤（primary female genital system lymphoma,PFGSL）是女性生殖系统少见的恶性肿瘤,其发病率低,临床报道较少。探讨PFGSL的临床特征、诊治及预后。方法：回顾分析2008年1月—2019年12月期间于复旦大学附属肿瘤医院就诊的57例PFGSL患者的临床病理学资料。57例患者均符合PFGSL诊断标准,其中单一治疗组33例,综合治疗组24例。中位随访时间37.5个月（2.0~85.0个月）。病灶位于宫颈的40例（70.2%）、卵巢13例（22.8%）、子宫3例（5.3%）、阴道1例（1.8%）。按国际预后指数（International Prognostic Index,IPI）评分,低危组36例（63.2%）,中危组21例（36.8%）。按淋巴瘤分期标准,ⅠE期36例（63.2%）,ⅡE期19例（33.3%）,ⅢE期2例（3.5%）。病理学类型为弥漫大B细胞淋巴瘤的患者有42例（73.7%）。结果：57例患者中,复发14例,复发率为24.6%（14/57）,其中12例位于单一治疗组,2例位于综合治疗组。其中复发位于中枢神经系统者4例,眼1例;局部复发者4例,全身多处转移者5例。57例患者的3年总生存（overall survival,OS）率为82%,5年OS率为78%;3年无病生存（disease-free survival,DFS）率为77%,5年DFS率为71%。单一治疗组复发率为36.4%（12/33）,3年OS率为72%,5年O率为64%;3年DFS率为67%,5年DFS率为57%。综合治疗组复发率仅为8.3%（2/24）,3、5年OS率为95%;3、5年DFS率为为91%。多因素分析显示,患者的OS率及DFS率均与治疗方式明显相关（P＜0.05)。结论：原发女性生殖系统淋巴瘤发病率低,临床表现无特异性,极易造成误诊,妇科医师应提高对此病的认识,并参考放化疗科医师意见进行诊治。

Clinical analysis of 57 cases of primary female genital system lymphoma

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 nervoussystem, 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.