原发女性生殖系统淋巴瘤临床分析

目的 探讨原发性女性生殖系统淋巴瘤(primary female genital system lymphoma,PFGSL)的临床和病理特征,分析诊断治疗方法及影响预后的因素。方法 回顾性收集2010年1月至2020年12月首都医科大学附属北京妇产医院初诊8例PFGSL患者的临床资料并随访。结果 患者平均发病年龄(54.3±12.6)岁,其中原发于卵巢4例,宫体2例,宫颈1例,外阴1例。主要临床表现为绝经后阴道出血、腹胀、腹水及盆腔肿物。病理类型均为B细胞来源的非霍奇金淋巴瘤(non-Hodgkin's lymphoma, NHL),免疫组织化学中CD20均呈阳性,Ki67较高。II_EA期患者4例,IV_A期患者3例,IV_B期患者1例。6例患者依据手术后病理诊断,2例患者行肿物活检诊断;5例患者接受了以CHOP方案为主的化学药物治疗(以下简称化疗),其中1例进行了自体干细胞移植。1例失访,2例死亡,5例生存(其中4例无瘤生存)。结论 PFGSL发病率低,临床表现无特异性,与原发妇科恶性肿瘤容易混淆。当肿物呈乳白色细腻样或反复抽取腹水无法找到癌细胞时需警惕PFGSL。活检或手术切除肿物可获得病理明确诊断,CHOP为主的化疗方案是其主要治疗手段。

Clinical analysis of primary female genital system lymphoma

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.