原发性乳腺弥漫性大B细胞淋巴瘤7例临床病理及预后分析

Primary breast diffuse large B-cell lymphoma: a clinicopathologic study and prognosis analysis of seven cases

目的：探讨原发性乳腺弥漫性大B细胞淋巴瘤（ diffuse large B-cell lymphoma, DLBCL）的临床病理学特征、诊断及鉴别诊断、治疗与预后。方法收集7例原发性乳腺DLBCL,观察其临床病理学特征及免疫表型,分析其治疗方案及预后因素。结果7例均为女性,年龄28-75岁,中位年龄51岁；左乳5例,右乳2例。乳腺及同侧腋窝无痛性包块是最常见的临床表现。镜下见肿瘤细胞中等大小或偏大,弥漫一致浸润分布于乳腺小叶间、导管周围、间质及脂肪组织中,部分呈单行条索状排列。1例为ALK-1阳性DLBCL,6例为DLBCL,非特殊类型。免疫表型分型：5例为非生发中心型,2例为生发中心型；Ki-67增殖指数60%-95%；Ann Arbor分期：I EA期2例,II EA期5例；IPI评分：4例0分,3例1分。7例患者中1例行改良根治术,4例行包块切除术,2例行空芯针穿刺活检病理诊断。2例未行化疗而死亡,5例行4-6周期（ CHOP/R-CHOP）化疗或联合放疗,4例获得完全缓解（ complete response, CR）,1例进展死亡。随访1-114个月,中位随访时间18个月,1年总生存期（ overal survival, OS）为57．1%,5年OS为14．3%。结论原发性乳腺DLBCL诊断主要依靠病理活检及免疫表型,免疫表型分型以非生发中心型为主,治疗以放、化疗等综合治疗方案为宜,预后需多因素综合评价。

Purpose To discuss the clinical pathological features, diagnosis and differential diagnosis, treatment and prognosis of pri-mary breast diffuse large B-cell lymphoma （ DLBCL） . Methods 7 cases of primary breast DLBCL were collected, their clinical path-ological characteristics and immunophenotypes were also observed, and the treatment and prognostic factors were discussed. Results All of the 7 patients were women, aged 28-75 years, with the median 51 years. 5 cases involved left breast, 2 cases were located in the right breast. Painless mammary mass and ipsilateral axillary lymphadenopathy were the commonest clinical manifestations. On mi-croscopic observation, tumor cells were large to medium-sized which characterized as diffuse infiltration between the lobules of mamma-ry gland, around the duct, interstitial and fat tissue, some were single file cord pattern. 1 case was ALK-1 positive DLBCL, 6 cases were DLBCL of the non special type. The immunophenotype showed 5 cases were of non-GCB, 2 cases were of GCB type. Ki-67 index were from 60% to 95%. According to Ann Arbor staging, 2 cases were stage I EA, 5 cases were stage II EA. IPI score：4 cases with 0 score, 3 cases with 1 score. Of 7 patients, one case was modified radical mastectomy, 4 cases were lumpectomy, 2 cases were diag-nosed by core needle biopsy. 2 patients died without chemotherapy, 5 patients were chemotherapy or radiotherapy for 4 to 6 cycles （ CHOP/R-CHOP） , 4 patients received complete response, one died. Patients were followed up from 1 to 114 months, the median fol-low-up time was 18 months. The 1 year OS rate was 57. 1%, 5 years OS rate was 14. 3%. Conclusion The diagnosis of primary breast DLBCL is confirmed by pathological biopsy and immunohistochemical markers. The immunophenotype was mainly non-GCB type. Comprehensive treatment including chemotherapy and radiotherapy is appropriate. The prognosis should be comprehensively eval-uated by multiple factors.