涎腺原发性MALT淋巴瘤5例临床病理分析

目的 探讨涎腺原发性结外边缘区黏膜相关淋巴组织(MALT)淋巴瘤的临床病理特征。方法 回顾性分析5例涎腺MALT淋巴瘤的临床病理资料，并复习相关文献。结果 5例患者中，女性3例，男性2例，发病年龄32～71岁，肿块位于单侧腮腺/颌下腺。肿瘤细胞主要由多种形态的肿瘤性边缘区B细胞构成，弥漫或结节状浸润生长，见“滤泡植入”、“淋巴上皮病变”伴导管内聚集生长、伴导管上皮周围基底膜样嗜酸性物质增多，见脉管、神经侵犯。肿瘤细胞B细胞相关抗原弥漫阳性，CD5、CD23、Cyclin D1均阴性，Ki-67增殖指数10%～30%。随访8～97个月，5例均未行放化疗，3例无复发，1例继发右上腭MALT淋巴瘤，1例死亡。结论 涎腺MALT淋巴瘤具有一定的形态学特征，呈B细胞免疫表型且无其他小B细胞淋巴瘤的表型特征。本病呈惰性进程，预后一般较好，可累及周围淋巴结及其他涎腺腺体，有复发和远处播散的风险，需要长期密切随访。     

眼睑黏膜相关性淋巴组织结外边缘区B细胞淋巴瘤二例并文献复习

目的 探讨眼睑黏膜相关性淋巴组织结外边缘区B细胞淋巴瘤（MALT淋巴瘤）的临床病理特征、诊断与鉴别诊断、治疗及预后。方法 回顾性分析哈尔滨市第一医院2例眼睑MALT淋巴瘤的临床资料、组织病理学形态和免疫组织化学结果,并复习文献。结果 2例患者以眼睑肿物为首发症状,均在局部麻醉下完成眼睑肿物切除术,病理检查示肿瘤细胞形态较一致,中等大小,胞质丰富,呈弥漫性或结节状排列,诊断为眼睑MALT淋巴瘤。免疫组织化学示2例患者肿瘤细胞均CD20（＋）,CD79a（＋）,CD3（-）,CD10（-）,bcl-6（-）,CD5（-）,cyclin D1（-）,CK（-）。结论 眼睑MALT淋巴瘤临床表现缺乏特异性,病因与感染有关,目前尚无一致的治疗方案,要避免过度治疗;病理学检查及免疫组织化学对该病的诊断和鉴别诊断具有重要意义。     

原发性胸腺结外边缘区黏膜相关B细胞淋巴瘤的临床病理分析

[目的]探讨原发性胸腺结外边缘区黏膜相关B细胞淋巴瘤的临床病理学特征、诊断及鉴别诊断.[方法]分析5例胸腺黏膜相关淋巴瘤的临床表现,对手术切除标本进行病理学和免疫组织化学(EnVision法)染色观察,并用PCR检测其中3例患者的免疫球蛋白重链(IgH)基因重排情况.[结果]5例均发生于成年女性,平均年龄50.4岁.其中3例既往有类风湿性关节炎病史,2例有干燥综合征病史.5例病例均行纵隔肿块切除,其中例4术后进行了化疗,目前所有病例随访均存活.大体均为囊实性肿块,光镜下胸腺正常结构消失,中心细胞样淋巴细胞弥漫浸润,并可见反应性淋巴滤泡形成.肿瘤内可见多个内衬上皮的囊腔形成,腔内可见大量红染无结构物质及胆固醇结晶.肿瘤性淋巴细胞浸润囊肿内衬上皮,形成明显的淋巴上皮病变.免疫组织化学检测显示,瘤细胞阳性表达CD20、CD79a、Bcl-2.而CK、CD3、CD5、CD23、CD10、Bcl-6、cyclinD1及TdT等标记均为阴性.3例PCR均检测出免疫球蛋白重链(IgH)基因克隆性重排.[结论]原发性胸腺结外边缘区黏膜相关B细胞淋巴瘤是一种非常少见的肿瘤,常发生在伴有自身免疫性疾病的成年女性中,大体上常呈囊实性,应与多房胸腺囊肿、胸腺淋巴组织增生及胸腺瘤等鉴别.     

肾脏黏膜相关淋巴瘤2例报道并文献回顾

背景与目的：原发肾脏的黏膜相关组织淋巴瘤十分罕见,目前世界报道不足50例。本文介绍2例肾脏黏膜相关淋巴瘤的形态学特点和免疫表型特征,旨在使临床和病理对这种低度恶性的B细胞肿瘤的特点有所了解。方法：收集病史资料,形态学评价根据HE切片,用免疫组化法检测肿瘤细胞的表型,使用的抗体包括CD20、CD79、CD5、CD10、CD43、CD23、BCL10和Cyc linD1。结果：2例患者均为女性,年龄分别为48岁和55岁,临床上均有慢性肾盂肾炎病史。B超和CT检查发现肾脏肿块,行全肾切除。大体检查可见肿块位于肾髓质,呈境界不清的暗红色;镜检见肾盂至肾实质弥漫淋巴样细胞浸润,以小淋巴细胞、中心细胞样细胞、淋巴浆细胞和浆细胞浸润为主,可见肿瘤细胞浸润肾小管和肾球囊形成淋巴上皮病变和反应性淋巴滤泡,但没有显著滤泡殖入;免疫组化显示增生细胞以B淋巴细胞为主,散在分布反应性T细胞,肿瘤细胞CD20、CD79 a阳性,CD43弱阳性,CD5、CD10、BCL10、CD23和Cyc linD1均为阴性。结论：原发肾脏黏膜相关淋巴瘤临床极为罕见,临床表现和辅助检查与肾细胞癌不易鉴别,但组织学特点符合经典黏膜相关淋巴瘤的所有特征,免疫表型有助于病理诊断。     

肺黏膜相关淋巴组织淋巴瘤临床病理观察

目的:探讨肺黏膜相关淋巴组织淋巴瘤的临床病理特点及诊断与鉴别诊断。方法:对4例肺黏膜相关淋巴组织淋巴瘤的临床资料、组织学形态及免疫组化结果进行分析。结果:肿瘤大体呈结节状,镜下见边缘区B细胞结节样增生,并向周围肺泡和肺泡间质弥漫性浸润,形成特征性的飘带样和串珠样结构。免疫组化标记,肿瘤细胞CD20、CD79a、Igλ、BCL-10阳性,CD3、CD45RO、CD5、CD23、CyclinD1、CD10、BCL-2阴性。结论:肺的黏膜相关淋巴组织淋巴瘤较为少见,组织形态特征是其诊断的主要依据,免疫组化有助于鉴别诊断。     

肺黏膜相关淋巴组织淋巴瘤临床病理观察

目的：探讨肺黏膜相关淋巴组织淋巴瘤的临床病理特点及诊断与鉴别诊断。方法：对4例肺黏膜相关淋巴组织淋巴瘤的临床资料、组织学形态及免疫组化结果进行分析。结果：肿瘤大体呈结节状,镜下见边缘区B细胞结节样增生,并向周围肺泡和肺泡间质弥漫性浸润,形成特征性的飘带样和串珠样结构。免疫组化标记,肿瘤细胞CD20、CD79a、Igλ、BCL-10阳性,CD3、CD45RO、CD5、CD23、CyclinD1、CD10、BCL-2阴性。结论：肺的黏膜相关淋巴组织淋巴瘤较为少见,组织形态特征是其诊断的主要依据,免疫组化有助于鉴别诊断。     

腰椎原发性前B淋巴母细胞性淋巴瘤1例报道并文献复习

目的:报道1例腰椎原发性前B淋巴母细胞性淋巴瘤并探讨其临床病理特点、诊断和鉴别诊断。方法:对1例腰椎原发性前B淋巴母细胞性淋巴瘤进行光镜、免疫组化检测和基因重排,并结合文献讨论。结果:镜下肿瘤细胞弥漫分布,细胞大小不一,胞质较丰富,核染色质呈粉尘状。免疫表型:肿瘤细胞CD99、LCA、CD20、CD79a弥漫阳性,CD3灶性阳性,VIM肿瘤细胞阴性,间质阳性,Ki-67增值指数为37%。结论:前B淋巴母细胞性淋巴瘤是一种罕见的高度恶性肿瘤,诊断和鉴别诊断主要依赖其病理形态学特点结合免疫表型。     

鼻腔人免疫缺陷病毒相关浆母细胞淋巴瘤临床病理分析

目的：探讨鼻腔人免疫缺陷病毒相关浆母细胞淋巴瘤的临床病理特点、诊断及鉴别诊断。方法应用光学显微镜观察、免疫组织化学染色、原位杂交法对1例人免疫缺陷病毒阳性原发鼻腔的浆母细胞淋巴瘤（PBL）患者肿瘤组织进行临床病理学检查,并复习相关文献。结果肿瘤组织弥漫性浸润,肿瘤细胞较大,圆形或椭圆形,胞质丰富,细胞核偏位,可见1～2个核仁,核分裂象易见,可见凋亡小体及星空现象。肿瘤细胞CD138、VS38c、CD79a弥漫阳性,CD20、EMA部分阳性,Ki-67阳性指数80%,κ轻链基因单克隆性重排,原位杂交检测EB病毒编码mRNA（EBER）阳性。结论原发鼻腔的浆母细胞淋巴瘤是一种罕见的弥漫增生的恶性肿瘤,肿瘤细胞形态类似B免疫母细胞,但是肿瘤细胞具有浆细胞的免疫表型。     

乳腺原发性非霍奇金淋巴瘤临床病理分析

目的：报道5例乳腺原发性非霍奇金淋巴瘤（PBNHL）探讨其临床病理特点、诊断和鉴别诊断。方法：对5例乳腺原发性非霍奇金淋巴瘤进行光镜、免疫组化和基因重排检测。结果：镜下肿瘤细胞均弥漫分布，2例瘤细胞中等大，圆形、卵圆形，胞质中等量，核大，染色质细，核仁不明显；2例瘤细胞呈空泡状，核仁明显；2例瘤细胞内可见浆细胞样淋巴细胞，5例核分裂均多见。免疫表型：4例肿瘤细胞均CD20、CD79a和LCA弥漫阳性，CD3和CD45RO灶性阳性。1例仅LCA阳性，而CD20、CD79a、CD3和CD45RO均阴性。结论：乳腺原发性非霍奇金淋巴瘤是一种较罕见的结外恶性淋巴瘤，诊断和鉴别诊断主要依赖其病理形态学特点并结合免疫表型。     

垂体原发性淋巴瘤 1 例并文献复习简

目的：报道1例原发于垂体的罕见的弥漫大B细胞淋巴瘤并结合文献探讨其临床病理特点、诊断和鉴别诊断。方法：对1例垂体原发性弥漫大B细胞淋巴瘤进行常规HE染色及免疫组化染色,分析其组织学特点及免疫表型。结果：镜下肿瘤由排列呈片状的小至中等大小的细胞构成,于蝶窦黏膜内弥漫性生长。瘤细胞形态较一致,圆形,核大、深染,异型性明显,染色质粗块状,核分裂多见,胞浆稀少。免疫表型：肿瘤细胞LCA、CD20、CD79α弥漫阳性,CD99弱阳性,FSH、LH、ACTH、GH、Pro、TSH、AE1／AE3、EMA、CD3、CD45RO、CD117、PLAP均阴性,Ki-67显示肿瘤细胞增殖指数约80％。结论：垂体原发性淋巴瘤（PPL）是一种非常罕见的恶性肿瘤,诊断和鉴别诊断主要依赖病理形态学特点和免疫表型。     

Focal Lymphoid Hyperplasia of the Terminal Ileum Presenting Mantle Zone Hyperplasia with Clear Cytoplasm. A Report of Three Cases

We report three unusual cases of focal lymphoid hyperplasia of the ileocecal valve. The gross specimens showed thickening of the ileocecal valve. Low power magnification showed a dense lymphoid infiltrate in the mucosa and submucosa. This condition was characterized by reactive lymphoid follicles with large reactive germinal centers surrounded by a pale cuff of mantle zone lymphocytes presenting a marginal zone distribution pattern. These cells had intermediate- to- medium-sized round or slightly indented nuclei and a broad rim of clear cytoplasm. However, immunohistochemical study demonstrated that both the mantle zone lymphocytes and the pale cuff of the lymphoid cells were CD20+, sIgM+, sIgD+, CD5−, CD10−, CD23−, CD43−, Bcl-2+, Bcl-6−, CyclinD1−. The polytypic nature of these cells was demonstrated by immunohistochemistry and polymerase chain reaction. This unusual mantle cell hyperplasia with clear cytoplasm associated with focal lymphoid hyperplasia in middle-aged and elderly patients should be differentiated from the extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue type or mantle cell lymphoma showing a marginal zone distribution pattern. To avoid overdiagnosis and overtreatment, it is suggested that immunophenotypic and genotypic studies might be required, and careful attention should be paid to the morphologic examination.     

Bilateral primary orbital non-Hodgkin's lymphoma in a patient with scleroderma: a case report

Orbital presentation of non-Hodgkin's Lymphoma (NHL) is uncommon but occurs both as the only site of disease and as a site of recurrence. Primary orbital NHLs are usually low-grade, and mostly extranodal marginal zone/mucosa associated lymphoid tissue B-cell neoplasms. They are commonly associated with chronic inflammatory conditions. The issue of bilaterality in orbital lymphoma is not very common. The onset of malignant lymphoproliferation may precede, follow, or exist simultaneously with scleroderma. Different treatment modalities were applied in orbital NHL such as surgery, radiation therapy, chemotherapy or both. We report a 57-year-old man with scleroderma presenting with eyelid hernias who had biopsy-proven marginal zone NHL, successfully treated with radiotherapy and combined chemotherapy, and also review the literature.     

Marginal zone lymphomas

In the World Health Organization classification of tumors of hematopoietic and lymphoid tissues, the group of marginal zone lymphomas comprises three different entities, namely the extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, the nodal marginal zone B-cell, and the splenic marginal zone B-cell lymphoma (with or without circulating villous lymphocytes). This article addresses each of the marginal zone lymphomas.     

Primary Dural Mucosa-Associated Lymphoid Tissue-Type Lymphoma: Case Report and Review of the Literature

A 47-year-old Hispanic male presented with visual field disturbances, memory impairment, and a seizure. CT and MRI were consistent with meningioma. Both neurologic exam and routine laboratory tests were within normal limits. The patient underwent craniotomy and subtotal resection of the tumor. On H&E, the lesion was composed of a lymphoid mass with well-defined irregularly shaped follicles surrounded by a monomorphic population of small lymphocytes. Marginal zones stained for B-cell markers, CD20 and CD79a, one T-cell marker, CD43, and kappa light chains. While other markers did not stain the majority of tumor cells, they did identify other lymphoid and plasma cell elements. A diagnosis of marginal zone B-cell lymphoma of dura, mucosa-associated lymphoid tissue (MALT)-type (extranodal) was made. MALT-type lymphomas are unusual in the nervous system; this is the first such case reported in a male and serves to emphasize the wide diversity of presentation of a neoplasm originally described in the GI tract and thus far described in the CNS only in females.     

BIOMED-2体系检测免疫球蛋白重链基因重排在黏膜相关淋巴组织结外边缘区B细胞淋巴瘤诊断中的应用

 【摘要】　目的　探讨BIOMED-2标准化体系检测免疫球蛋白重链（IGH）基因重排对黏膜相关淋巴组织结外边缘区B细胞淋巴瘤（MALT淋巴瘤）诊断的应用价值。方法　45例标本包括不同部位原发的MALT淋巴瘤36例、淋巴结外淋巴组织增生性病变3例、幽门螺杆菌（HP）感染相关性重度胃炎6例。用DNA提取试剂盒从石蜡包埋组织中提取所选病例的基因组DNA,通过BIOMED-2体系质控混合引物检测其质量,应用IGH VH-JH 3组引物组合进行IGH基因克隆性重排检测;比较分析该体系在MALT淋巴瘤诊断中的敏感性和特异性。结果　45例中31例（MALT淋巴瘤22例,淋巴组织增生性病变3例、重度胃炎6例）提取的基因组DNA可扩增出300 bp片段,其余14例DNA均严重降解。22例MALT淋巴瘤中,16例检测出IGH基因重排,灵敏度为72.7 %;6例严重胃炎病例未检测出IGH克隆性重排,其特异度是100 %;3例淋巴组织增生性病变中1例检测出IGH克隆性重排,2例未检测出重排。结论　在MALT淋巴瘤的诊断和淋巴组织增生性疾病的鉴别诊断中,BIOMED-2标准化体系检测IGH基因克隆性重排是一种快速可靠的方法,具有重要的临床应用价值。     

肺MALT淋巴瘤并腮腺淋巴上皮病1例及文献复习

目的：探讨肺黏膜相关淋巴组织型边缘区B细胞淋巴瘤（MALT-MZL）的临床表现、影像和病理学特点、治疗方法及其与淋巴上皮病（LEL）的关系.方法：报道1例肺MALT淋巴瘤并腮腺淋巴上皮病,对其进行病理组织学观察和免疫组织化检测,并回顾文献.结果：肺MALT淋巴瘤无明显临床表现,影像学特点为边界不清斑片影、结节影、团块影和叶段实变影,可见支气管充气征,缺乏特异性.病理示小淋巴细胞弥漫浸润,支气管、细支气管和肺泡上皮的浸润（淋巴上皮病变）是特征性的,但不特异.免疫组化可显示肿瘤的单克隆性,CD20和CD79a阳性.腮腺LEL是MALT淋巴瘤前期病变,能恶变为MALT淋巴瘤.结论：肺MALT淋巴瘤是一种罕见肿瘤,临床无特异性,确诊靠病理组织学和免疫组化检查,治疗以手术和化疗为主.LEL能恶变为MALT淋巴瘤.     

Primary gastric mantle cell lymphoma in a patient with long standing history of Crohn's disease

The stomach is the most common site of primary extranodal lymphoma. Virtually all cases are of B-cell lineage, including extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT lymphoma) and diffuse large B-cell lymphomas. While secondary gastric involvement from nodal mantle cell lymphoma (MCL) or in the course of primary intestinal MCL (lymphomatous polyposis) have been described, primary gastric MCL has not been reported so far. A 74-year-old man with a 14 year-history of Crohn's disease was admitted at a general hospital due to epigastric pain refractory to therapy with proton-pump inhibitors. Endoscopy disclosed a large polypoid tumor with an ulcerated surface at the greater curvature of the gastric antrum. Endosonography demonstrated the tumor to be limited to the stomach with only local lymph node involvement. Histology of gastric biopsies revealed a dense atypical lymphoid infiltrate composed of small to medium sized cells with slightly irregular nuclear contours. Immunohistochemichally, the cells were positive for CD20, CD79a, CD43 and cyclin D1, but negative for CD3, CD5 and bcl-6. They stained for IgM and showed lambda-light chain restriction. Fluorescent in situ hybridisation studies showed the presence of the t(11;14) characteristic for MCL. No further evidence of lymphoma was found on extensive clinical staging. Following chemotherapy the patient is disease free at 24 months after diagnosis. This is the first case of a primary localized gastric MCL. The lack of CD5 expression underscores the importance of performing thorough immunohistochemical studies, particularly to exclude MALT lymphoma.     

Gastric MALT lymphoma treated with primary radiotherapy in the setting of autoimmune disease

Autoimmune and microbial-induced immune reactions are associated with lymphomagenesis, particularly of extranodal marginal zone B-cell lymphomas. This report presents a case of Helicobacter pylori-negative gastric extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) in the setting of Sj?gren syndrome and Hashimoto thyroiditis treated with primary involved-field radiotherapy. This report describes etiologic data, diagnostic and treatment considerations, and sophisticated radiation therapy techniques aimed at reducing long-term toxicity in this indolent disease.     

Isolated B-cell lymphoproliferative disorder at the dura mater with B-cell chronic lymphocytic leukemia immunophenotype

Lymphoma manifestations of the dura mater are extremely rare and have mostly been attributed to extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type. We report a patient with an isolated meningeal tumor, identified as a B-cell lymphoproliferative disorder with typical B-cell chronic lymphocytic leukemia immunophenotype. Because of the subclinical detection of trisomy 3 in the bone marrow by cytogenetic analysis and interphase fluorescence in situ hybridization, CD5(+) MALT is an important differential diagnosis; however, to our knowledge, this entity has never been reported in the context of dural lymphoma.     

Primary mucosa-associated lymphoid tissue lymphoma of the breast

Mucosa-associated lymphoid tissue (MALT) lymphoma is an extranodal indolent lymphoma with histopatholigic features similar to those of marginal zone B-cell lymphomas. Primary breast MALT lymphomas were first described by Lamovec and Jancar as a low-grade B-cell lymphoma in 1987. Herein, a case is presented of a patient with primary MALT lymphoma of the breast. Issues in diagnosis and breast-conservation treatment, as it pertains to primary MALT lymphoma of the breast, will be discussed.