间变性浆细胞肿瘤21例临床病理学分析  

Aanaplastic plasma cell neoplasms: a clinicopathological analysis of 21 patients

作　　者：郑重[1] 李志文[1] 史倩芸 何璐 吴鸿雁[1] 陈洁宇[1] 樊祥山[1] ZHENG Zhong;LI Zhi-wen;SHI Qian-yun;HE Lu;WU Hong-yan;CHEN Jie-yu;FAN Xiang-shan(Department of Pathology, the Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing 210008, China)

机构地区：[1]南京大学医学院附属鼓楼医院病理科,南京210008

出　　处：《临床与实验病理学杂志》2022年第3期313-318,共6页Chinese Journal of Clinical and Experimental Pathology

基　　金：南京市医学科技发展项目(QRX17004、YKK15058)。

摘　　要：目的探讨间变性浆细胞肿瘤(anaplastic plasma cell tumors,APCN)的临床病理学特征、诊断及鉴别诊断和预后。方法收集21例APCN的临床资料,分析其临床特点、组织学特征、免疫表型及分子病理学改变,并复习相关文献。结果21例APCN中男性15例,女性6例,平均年龄63岁(26~92岁)。其中11例为浆细胞骨髓瘤,主要表现为多发性骨质破坏伴软组织影,骨髓活检显示浆细胞异常增生,血清学检查可见单克隆免疫球蛋白;8例为骨外孤立性浆细胞瘤,临床表现为局部软组织肿块,主要发生于鼻腔鼻咽等部位;2例为骨的孤立性浆细胞瘤,发生在枕骨和椎骨。组织学上肿瘤细胞呈弥漫片状分布;细胞大小不一,呈圆形或不规则形,胞质嗜碱性;细胞核偏位或呈分叶状,可见粗大的染色质团块及多核细胞,核仁明显,并可见核内包涵体;核分裂象和凋亡坏死易见;此外可出现成熟性浆细胞瘤样分化的肿瘤细胞。免疫表型:肿瘤细胞CD38、CD138和MUM1均阳性,CD79a部分阳性,CD20均阴性;19例免疫球蛋白多呈轻链限制性,其中κ链11例,λ链8例;Ki-67增殖指数20%~80%。3例鼻腔鼻咽部APCN肿瘤细胞EBER原位杂交呈阳性。分子检测:主要出现IgH基因重排和1q21基因扩增。治疗:间变性浆细胞骨髓瘤多采用联合化疗;骨和骨外孤立性浆细胞瘤主要为手术治疗和(或)放疗。12例患者获得随访资料,其中6例死亡,2例复发,4例无瘤存活。结论APCN较为罕见,其临床表现多样,形态学多形性明显,需与具有多形性组织学形态的肿瘤相鉴别,其治疗主要采用联合化疗或放疗,预后较差。Purpose To study the clinicopathological features,diagnosis,differential diagnosis and prognosis of anaplastic plasma cell tumors(APCN).Methods 21 cases of APCN were studied by the clinical features,histological features,immunophenotypic and molecular pathological changes,with the relevant literature was reviewed.Results APCN was commonly seen in middle and elderly-aged men,with an average age of 63 years(26-92 years).Among them,11 cases were plasma cell myeloma,mainly manifested as multiple bone destruction with soft tissue shadow,bone marrow biopsy showed abnormal proliferation of plasma cells.Serological examination revealed a monoclonal immunoglobulin in serum.8 cases were extraosseous solitary plasmacytoma and showed focal soft tissue masses,which mainly occurred in the nasal cavity,nasopharynx.2 cases were solitary plasmacytoma of bone,which occurred in occipital bone and vertebra.Histologically,the tumor cells were uneven in size,round or irregular with basophilic cytoplasm,the nuclei were biased or lobated,with clump coarse chromatin and mutincleated cells with prominent nucleoli and intranuclear inclusions.In addition,mitosis,apoptosis and necrosis were easily seen.Furthermore,tumor cells with mature plasmacytoma-like differentiation could be observed.Immunohistochemical examination revealed tumor cells were positive for CD38,CD138,MUM1 and negative for CD20 in all cases,and CD79a was positive in some cases.Immunoglobulins mostly showed light chain restriction,among them 11 cases were kappa light chain and 8 cases were lambda light chain,the value of MIB1-labeling index was 20%-80%.It should be noted,EBER in situ hybridization was positive in 3 cases of APCN,which occurred in nasal cavity and nasopharynx.IgH gene rearrangement and 1q21 gene amplification mainly were found by molecular detection method.The patients who suffers from APCN were mostly treated with combined chemotherapy,solitary plasmacytomas of bone and extraosseous were treated with surgery and/or radiotherapy.Follow up data were obtained i

关 键 词：浆细胞肿瘤 浆细胞骨髓瘤 孤立性浆细胞瘤 间变性 临床病理 

分 类 号：R733.3[医药卫生—肿瘤] R733.4[医药卫生—临床医学]