目的 探讨血管内大B细胞淋巴瘤(intravascular large B-cell lymphoma,IVLBCL)及其碰撞瘤的临床病理特征、诊断及鉴别诊断.方法 收集徐州医科大学附属医院病理科(3例)、山东大学齐鲁医院病理科(1例)、西安交通大学第一附属医院病理科(1例)诊断的5例IVLBCL,其中2例为碰撞瘤,1例合并肝硬化,分析总结其形态及免疫表型特点,并复习相关文献.结果 5例患者年龄53～73岁(中位年龄65岁),男女比3∶2.肿瘤分别位于双下肢、右侧小脑半球、左肾、双侧鼻腔及肝脏,其中例2和例3分别因脑膜肿瘤、肾癌手术而偶然发现于脑膜瘤、透明细胞性肾细胞癌组织中,为碰撞瘤;例5发生于肝硬化基础上.形态上异型淋巴细胞均位于小血管和毛细血管腔内,细胞体积大、胞质少、核染色深,可见明显核仁及核分裂象.免疫组织化学瘤细胞均表达CD20、PAX5,2例CD5阳性;1例为生发中心B细胞(GCB)表型,4例为非GCB表型;C-MYC均阳性表达(阳性率10％～40％),4例PD-L1阳性(阳性率60％～90％);Ki-67阳性指数70％～90％;广谱细胞角蛋白(CKpan)、CD3、末端脱氧核苷酸转移酶(TDT)、CD34均阴性.例2脑膜瘤细胞孕激素受体(PR)、上皮细胞膜抗原(EMA)、波形蛋白阳性,CKpan、PD-L1阴性;例3肾透明细胞癌细胞CKpan、PAX8、EMA、波形蛋白、碳酸酐酶Ⅸ(CAⅨ)、CD10均阳性,PD-L1阴性.5例原位杂交检测EB病毒编码的RNA均阴性,荧光原位杂交检测C-MYC基因均未见断裂.3例患者随访1～13个月,均死亡.结论 IVLBCL临床表现非特异,侵袭性高、预后差,该肿瘤的碰撞瘤极其罕见,病理医师应提高对其认识,行充分的鉴别诊断避免误诊及漏诊.
Clinicopathological features of intravascular large B-cell lymphoma and collision tumors of five cases
Objective To investigate the clinicopathological characteristics,diagnosis and differential diagnosis of intravascular large B-cell lymphoma(IVLBCL)and its collision tumors.Methods Five cases of IVLBCL were collected,including 2 cases of collision tumors,and 1 case complicated with liver cirrhosis.The morphology and immunophenotype were analyzed.The related literature was reviewed.Results There were 2 females and 3 males,aged from 53 to 73 years,with a median age of 65 years.The tumors were located in the lower extremities,right cerebellar hemisphere,left kidney,bilateral nasal cavity,and liver,respectively.Cases 2 and 3 were incidentally found in meningioma and renal cell carcinoma tissues,respectively.Case 5 had a background of liver cirrhosis.Morphologically,atypical large lymphoid cells were located in small blood vessels and capillary lumen,with little cytoplasm,hyperchromasia,prominent nucleoli,and obvious mitotic figures.Immunohistochemically,the IVLBCL tumor cells expressed CD20 and PAX5;2 cases were CD5 positive.One of the 5 cases was GCB phenotype,and 4 cases were non-GCB phenotype.All cases expressed C-MYC(positive rate was 10％-40％).PD-L1 was positive in 4 cases(positive rate was 60％-90％).Ki-67 proliferation index was 70％-90％.CKpan,CD3,TDT,and CD34 were negative.In case 2,meningioma cells were positive for PR,EMA,and vimentin,but negative for CKpan and PD-L1.In case 3,renal carcinoma cells were positive for CKpan,PAX8,EMA,vimentin,CAⅨ and CD10,while PD-L1 was negative.No EBER expression(by in situ hybridization)or C-MYC gene translocation(FISH,break-apart probe)was detected in any of the 5 cases.Three patients were followed up,and all died within 1-13 months.Conclusions IVLBCL is a highly aggressive lymphoma,with occult clinical manifestations and poor prognosis.Collision tumors of IVLBCL are extremely rare.A better understanding of IVLBCL would help pathologists avoid misdiagnoses.
NETL
NSTL
万方数据
维普
