鼻腔、鼻窦髓外浆细胞瘤的诊断与治疗(12例报告及文献分析)

目的 探讨鼻腔、鼻窦髓外浆细胞瘤的临床特点与患者预后,以提高对该病的诊断与治疗水平。方法 收集2000年1月～2010年11月住院确诊的12例鼻腔、鼻窦浆细胞瘤患者的病历及随访资料,就临床表现、诊断、治疗及预后进行回顾性分析。结果12例患者中,男9例,女3例,男女比3∶1; 35～61岁,平均49.6岁。12例患者均经手术治疗,病理确诊为髓外浆细胞瘤,术后放疗 10 例,术后放、化疗1 例。免疫组化染色排除多发性骨髓瘤,结果 显示CD38均为阳性,CD79a阳性5例,CD20、CD56及CyclD1均为阴性。随访1～59个月,平均随访（53±8）个月。术后1年6月及3年各失访1例,术后1年6月局部复发伴重症感染死亡1例,1例术后2 年3月转化为多发性骨髓瘤,再行化疗,术后4年死亡。结论 鼻腔、鼻窦髓外浆细胞瘤好发于中年男性,诊断主要依靠病理检查,手术+放疗预后较好,部分可转化为多发性骨髓瘤。     

鼻及鼻咽孤立性髓外浆细胞瘤的CT和MRI表现

目的探讨鼻及鼻咽孤立性髓外浆细胞瘤的CT和MRI表现,并评价两种影像学检查方法的临床应用价值。资料与方法回顾性分析8例经组织学证实鼻及鼻咽孤立性髓外浆细胞瘤患者的影像学资料。结果发生于鼻咽3例,上颌窦2例,鼻腔1例,鼻腔、筛窦及蝶窦1例,鼻腔、额窦、筛窦、蝶窦及上颌窦1例;病变边界较清楚,呈椭圆形3例,分叶状5例;病变最大径20～92mm,平均49mm。CT表现:鼻部病变造成周围骨质变形、变薄、移位、吸收,其中3例伴局部破坏;鼻咽病变位于黏膜下,黏膜表面光滑,邻近骨质未受累。与脑实质比较,平扫7例呈等密度,1例呈不均匀高密度;增强后2例呈中度、较均匀强化,1例呈不均匀高度强化。MRI表现:T1WI、T2WI均呈等信号,其中7例T1WI信号较均匀,T2WI散在细条状略高信号,另1例可见片状短T1、长T2出血信号;增强后病变中度强化6例,显著强化2例,其中7例病变内可见强化更明显的细条状间隔,对应T2WI所示略高信号。3例动态增强扫描的时间-信号强度曲线均为速升缓降型。MRI均清楚显示病变的范围、伴发的炎症及术后的复发。结论鼻部病变所致骨质变形和吸收、鼻咽病变黏膜下生长、MRI平扫等信号和增强后所示间隔强化是鼻及...     

神经系统髓外浆细胞瘤临床病理学观察

背景与目的：发生于神经系统的髓外浆细胞瘤罕见。本研究分析神经系统髓外浆细胞瘤的临床病理特点。方法：总结3例神经系统髓外浆细胞瘤临床病理特点,EnVision法分析免疫表型（LCA、CD20、CD79a、CD3、CD7、PC、MUM1、CD138、Ki-67）,PCR法分析IgH和TCRγ基因重排,并进行治疗随访。结果：3例神经系统髓外浆细胞瘤,2例发生于大脑,均为男性,年龄为48和64岁。1例发生于T10椎管内,女性,29岁。影像学均提示占位性病变,临床表现为头痛呕吐,病理学特征为：不同分化程度的浆细胞样肿瘤细胞弥漫性浸润,间质内血管丰富或形成血湖,可见淀粉样物质沉积。免疫表型：3例之瘤细胞均表达CD79a,PC和MUM1,均不表达CD20和CD3、CD7。基因重排分析结果显示IgH基因均呈单克隆性重排。结论：神经系统髓外浆细胞瘤具有特征性的临床病理学改变,免疫组化和基因重排分析能够帮助确定肿瘤细胞起源,诊断时应综合考虑,并排除多发性骨髓瘤。     

Overall Survival Trends and Clinical Characteristics of Plasmacytoma in the United States: A National Cancer Database Analysis

BackgroundGiven the rarity of plasmacytoma, large-scale database analysis can provide useful information regarding the clinical presentation and patient-related factors impacting overall survival (OS).Materials and MethodsThe National Cancer Data Base was queried for patients with plasmacytoma between 2004 and 2013, excluding patients with systemic disease. Plasmacytomas were classified as originating in bone (P-bone), in extramedullary tissue (P-EM), or unspecified. Survival was estimated using the Kaplan-Meier and log-rank test method. We used Cox regression to determine specific outcomes adjusting for demographic, socioeconomic, geographic, facility type, year of diagnosis, and comorbid factors.ResultsIn total, 6225 patients were identified, of which 61.5% were men. The median age at diagnosis was 64 years (range, 18-90 years), and the median follow-up was 58 months. The primary site of disease was P-bone in 4056 (65.1%) patients and P-EM in 1468 (23.6%), and the remaining 701 patients were P-unspecified. The unadjusted median survival for solitary P-bone was 89 months (95% confidence interval, 82.9-95.0 months), and for solitary P-EM was 117.3 months (95% confidence interval, 108.8 months to not reached). Factors associated with improved OS include younger age, private insurance, higher income, solitary lesion, and lower comorbidity score. Patients with P-bone disease treated at academic facilities had improved OS. Only 65% of patients with solitary plasmacytoma lesions received radiation treatment. Age greater than 75 years and increased distance to treatment facility was associated with a decreased likelihood of receiving radiation.ConclusionsThis is the largest study examining outcomes of patients with plasmacytoma using a large database analysis, revealing unique aspects of P-EM versus P-bone and underutilization of radiation treatment.     

母细胞性浆细胞样树状突细胞肿瘤1例报告并文献复习

目的:探讨母细胞性浆细胞样树状突细胞肿瘤的临床、组织病理学及免疫表型特征。方法:报道1例母细胞性浆细胞样树状突细胞肿瘤,并结合文献分析其临床病理特点。结果:该例以皮肤最先受累,后很快累及骨髓、淋巴结、外周血。组织学特点为真皮内肿瘤细胞弥漫性浸润。骨髓肿瘤细胞表达CD4和CD56。EB病毒阴性,T细胞受体基因重排阴性。结论:该病是一种罕见的侵袭性淋巴瘤,进展快,病死率高,易误诊。诊断依据临床和组织学特点,结合免疫组化标记、EB病毒和TCR克隆性重排检测等综合分析。     

A 3-way collision tumor of the upper respiratory tract: a composite of 2 immunophenotypically distinct mantle cell lymphomas and a plasmacytoma

Composite lymphoma (CL) is composed of 2 or more morphologically and immunophenotypically distinct lymphomas in a single anatomical site. Here we report a unique CL of the upper respiratory tract in an elderly male patient. Morphologically, the lymphoma was composed of 2 distinct and well-demarcated areas consisting of monotonous small to medium-sized lymphocytes and sheets of mature-appearing plasma cells. Immunophenotyping by both flow cytometry and immunohistochemistry revealed that the small to medium-sized lymphocytes were composed of 2 distinct subpopulations sharing a CD5(+)/CD19(+)/CD20(+)/CD22(+)/CD23(-)/FMC-7(+)/cyclin D1(+) immunophenotype but with different immunoglobulin (Ig) light and heavy chain expression, consistent with 2 immunophenotypically distinct mantle cell lymphomas (MCLs); the plasma cells were composed of CD38(bright +)/CD138(+)/IgG kappa-restricted plasma cells, consistent with a plasmacytoma. Fluorescence in situ hybridization showed the t(11;14) translocation present in the lymphocyte region but absent in the plasma cell area. Ig heavy chain gene rearrangement studies on manually dissected populations showed 2 distinct patterns for the MCL and plasmacytoma. To our knowledge, this is the first report of a 3-way CL consisting of 2 immunophenotypically distinct MCLs and a plasmacytoma.     

Extraosseous calcified plasmacytoma causing thoracic myelopathy

We report on a rare, calcified, plasma cell tumor of the spine causing progressive myelopathy. Other unusual features were the lack of an osseous lesion at the site of the mass, considerable calcified amyloid within the mass but no identifiable amyloid elsewhere, and normal serum immunoelectrophoresis. Received: 12 February 1999 Revision requested: 18 March 1999 Revision received: 19 April 1999 Accepted: 20 April 1999