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目的探讨原发性乳腺弥漫性大B细胞性淋巴瘤(DLBCL)的临床病理学特点。方法收集12例原发性乳腺DLBCL的病例标本,对其进行临床病理分析、免疫组化检测、EBER原位杂交及IGH、IGK和IGL基因重排检测。结果 12例均为女性,发病年龄22~77岁,平均年龄50.4岁,均表现为单侧乳腺肿块(左、右各6例)。组织学上,表现为成片的中等至大的肿瘤细胞弥漫浸润乳腺小叶、导管周围、间质及周围脂肪组织。根据免疫组化表型4例表现为弥漫性大B细胞淋巴瘤生发中心B细胞样型,8例表现为非生发中心B细胞样型,其中伴MYC和bcl-2或bcl-6双阳性5例;Ki-67增殖指数70%~90%。12例CD20和CD79α均(+),EBER原位杂交均为(-),基因重排12例IGH重排(+),6例IGK重排(+),1例IGL重排(+)。病例随访时间为12~87个月,随访时期内1例死于本病,2例带病生存,7例病情缓解无病生存,2例失访。结论乳腺原发性DLBCL是一种少见的恶性淋巴瘤,具有独特的临床病理特点,多为非生发中心B细胞样型,具有较高的增殖活性,预后差。
Objective To investigate the clinicopathological features of primary breast diffuse large B cell lymphoma (DLBCL). Methods A total of 12 cases of primary breast DLBCL were collected and analyzed by clinicopathological analysis, immunohistochemistry, EBER in situ hybridization and IGH, IGK and IGL gene rearrangements. Results All the 12 cases were female. The age of onset was from 22 to 77 years with a mean age of 50.4 years. All of them showed unilateral breast masses (left and right in 6 cases). Histologically, the medium to large tumor cells in the form of flakes diffusely infiltrate breast lobular, ductal, interstitial and surrounding adipose tissue. According to the immunohistochemical phenotype 4 cases showed diffuse large B cell lymphoma germinal center B cell type, 8 cases showed non-germinal center B cell type, which with MYC and bcl-2 or bcl-6 double positive 5 Cases; Ki-67 proliferation index of 70% to 90%. 12 cases of CD20 and CD79α (+), EBER in situ hybridization (-), 12 cases of gene rearrangement IGH rearrangement (+), 6 cases of IGK rearrangement (+), 1 case of IGL rearrangement (+). The follow-up time ranged from 12 to 87 months. One patient died of this disease during the follow-up period. Two patients survived. Seven patients were relieved of disease-free survival and two patients were lost to follow-up. Conclusion Primary DLBCL in breast is a rare malignant lymphoma with unique clinicopathological features, mostly non-germinal center B cell type with high proliferative activity and poor prognosis.