13例髓外浆细胞瘤临床病理特征分析

Clinicopathological Analysis of 13 Cases of Extramedullary Plasmacytoma

目的探讨髓外浆细胞瘤(EMP)的临床病理学特征及其鉴别诊断。方法运用组织病理学、免疫组织化学染色及原位杂交技术,回顾性分析13例EMP的临床病理特征,并结合相关文献进行讨论。结果13例患者中,男性10例,女性3例,发病年龄40~79岁,中位年龄65岁。其中鼻腔4例,肺3例,颈部淋巴结、鼻咽、喉、胃角、十二指肠及睾丸各1例。实验室检查无明显异常,骨髓穿刺未见明显改变,血免疫球蛋白检查正常,尿蛋白阴性,全身骨扫描未见明显异常改变。随访时间0.5~10.6 a。2例患者进展为多发性骨髓瘤,1例患者死于心血管疾病,其余患者均健在。镜下肿瘤细胞弥漫性生长,分化好者形态接近成熟的浆细胞,分化差者细胞异型性增大,核仁明显,似浆母或免疫母细胞样形态,核分裂象多见。免疫组化结果:13例均表达CD38、CD138和CD79a,8例表达MUM1,3例表达CD56和EMA,2例表达CyclinD1,1例表达CD20,1例表达CD10,KI67的增殖指数为3%~80%。轻连κ和λ表达情况为:6例表达λ,5例表达κ,2例κ和λ均阴性。结论EMP是一种罕见的原发浆细胞的淋巴造血系统肿瘤,中老年男性多见,预后较好,少数可转化为多发性骨髓瘤。诊断时需与浆细胞反应性增生、小B细胞淋巴瘤及侵袭性大细胞淋巴瘤、低分化癌等鉴别。

Objective To investigate the clinicopathological features and differential diagnosis of extramedullary plasmacytoma.Methods Histopathology,immunohistochemical staining and in situ hybridization were used to retrospectively analyze the pathological features of 13 cases of extramedullary plasmacytoma.It was discussed in combination with relevant literature.Results There were 10 males and 3 females in the 13 patients.The onset age was 40-79 years old,and the median age was 65 years old.There were 4 cases in nasal cavity and 3 cases in lung.Cervical lymph node,nasopharynx,larynx,gastric horn,duodenum and testis were 1 case respectively.There was no obvious abnormality in laboratory examination.Bone marrow puncture showed no obvious change.The blood immunoglobulin test was normal.The urine protein was negative.The whole body bone scan showed no obvious abnormal changes.The patients were followed up for 0.5-10.6 years.Two patients developed multiple myeloma.One patient died of cardiovascular disease,and the rest were alive.Light microscope showed diffuse growth of tumor cells.The morphology of well differentiated cells was similar to mature plasma cells.In poorly differentiated patients,the cell atypia increased,nucleolus obvious,which like plasmablastic or immunoblastic morphology,and nuclear division was common.Immunohistochemical results showed that 13 cases were positive for CD38,CD138 and CD79a,8 cases were positive for MUM1,3 cases were positive for CD56 and EMA,2 cases were positive for CyclinD1,1 case were positive for CD20,1 case were positive for CD10,and the proliferation index of Ki67 was 3%-80%.Lightκandλexpression showed thatλwas 6 cases,κwas cases,andκandλwere negative in 2 cases.Conclusion Extramedullary plasmacytoma is a rare primary plasma cell lymphoid hematopoietic system tumor,which is common in middle-aged and elderly men,and has a good prognosis.A few of them can be transformed into multiple myeloma.The differential diagnosis includes plasma cell reactive hyperplasia,small B-cell lymphoma,invasive large cell lymphoma and poorly differentiated carcinoma.