原发性肺大B细胞淋巴瘤的临床病理研究

目的探讨原发性肺弥漫性大B细胞淋巴瘤的组织学特点和免疫表形特征。方法运用组织形态学和免疫组织化学方法研究我院1例肺弥漫性大B细胞淋巴瘤患者的临床表现、病理特点和免疫组化标记特点。结果肉眼观察肿瘤为灰白色不规则分叶状团块,切面灰白色,鱼肉样,有大量坏死灶;显微镜观察被覆呼吸上皮下中心母细胞样细胞弥漫增生,浸润肺组织,瘤细胞坏死明显。免疫组化：CD20（＋）,CD79α（＋）,CD3（-）,CD45RO（-）,PCK（-）。结论弥漫性大B细胞淋巴瘤是一种少见的恶性肿瘤,而原发于肺的DLBCL更极为罕见。该病缺乏典型的临床表现,极易误诊。肺弥漫性大B细胞淋巴瘤的诊断和鉴别诊断,要以组织病理学形态为基础并与免疫组织化学相结合。     

直肠血管肉瘤1例

目的:探讨胃肠道血管肉瘤的临床病理特征及鉴别诊断及治疗预后.方法:在光学显微镜下对直肠血管肉瘤进行组织形态学观察,并借助免疫组织化学进一步对血管肉瘤的特点进行分析.由于累及直肠的血管肉瘤极为罕见,本研究对相关的文献资料进行回顾分析与总结.结果:累及直肠的血管肉瘤可以首先累及外膜或者先侵犯直肠黏膜.大体上为灰褐色结节状,伴有广泛的出血坏死,切面呈多彩状;显微镜下组织学表现为肿瘤细胞弥漫分布,形成大小不等互相吻合的管腔,免疫组织化学显示瘤细胞表达CD31和Vimentin等血管和间叶分化.肿瘤高度恶行.本例患者术后5 mo死亡.结论:血管肉瘤,特别是深部软组织及胃肠道的血管肉瘤,具有高度侵袭性并且快速转移,预后很差.在临床病理实践中,必须首先与常见的发生于该部位恶性肿瘤如癌,恶性黑色素瘤鉴别.     

心脏原发性血管肉瘤3例临床病理学观察

目的探讨心脏原发性血管肉瘤的临床病理学特征及鉴别诊断,提高对该肿瘤的认识。方法回顾性分析3例发生在心脏的血管肉瘤的临床资料、病理形态学、免疫组化结果,并复习相关文献。结果 2例患者为男性,1例患者为女性,年龄33~51岁;均发生在右心房,1例发生肺转移。临床表现无特异性,表现为胸闷、心慌、气促和呼吸困难。镜下瘤组织主要由梭形、卵圆形细胞构成,呈团块、条索状、乳头状结构排列或围成不规则、互相吻合的血管腔,出血及坏死多见。免疫组化显示肿瘤细胞:CD31(+),CD34(+),FⅧ(+)。结论心脏原发性血管肉瘤非常罕见,由于其临床表现无特异性,早期诊断困难,确诊主要依赖于病理组织学及免疫组化检查。     

低度恶性子宫内膜间质肉瘤14例病理特点分析

采用HE及免疫组织化学染色法分析14例低度恶性子宫内膜间质肉瘤(LGESS)的临床病理特点,结果HE染色示肿瘤组织成巢团样浸润,肿瘤细胞呈圆形、卵圆形或梭形,肿瘤内有大量小血管;免疫组织化学染色(8例)示CD10均阳性,角蛋白均阴性;4例波形蛋白阳性,3例ki-67抗体阳性指数<40%;2例伴性索样分化者ER、PR阳性,3例伴平滑肌分化者中2例SMA阳性,1例结蛋白阳性.提示LGESS确诊主要依靠组织病理学和免疫组织化学检查;CD10是一种比较特异的标记.     

原发性肝脏平滑肌肉瘤的病理及免疫组织化学观察

目的探讨原发性肝脏平滑肌肉瘤(primary hepatic leiomyosarcoma,PHLMS)的病理学及免疫组织化学表现。方法应用组织病理学、免疫组织化学方法观察3例原发性肝脏平滑肌肉瘤。结果组织学观察瘤细胞呈梭形,可见核分裂;免疫组织化学结果Smooth Muscle Actin( )、CD117(-)、S-100(-)、Vimentin(-)。结论原发性肝脏平滑肌肉瘤是一种罕见的恶性间叶肿瘤,其诊断主要依靠免疫组织化学方法。     

肝肉瘤样癌的临床病理特征

目的:探讨肝肉瘤样癌患者的临床病理学特征、诊断、鉴别诊断、治疗方法和预后.方法:以我院收治的1例肝肉瘤样癌患者为对象,分析其临床表现及诊疗经过,并进行随访,同时对送检标本进行病理学检查,结合有关文献探讨该病的临床病理学特点.结果:肿瘤位于肝脏右后叶,呈浸润性生长.组织学上可见上皮源性肿瘤成分与梭形细胞肉瘤成分同时存在并伴有移行现象;肿瘤中见大片凝固性坏死.免疫组织化学显示肿瘤细胞呈CK(Pan)和EMA强阳性,AFP和Hepar-1呈弱阳性表达,免疫组织化学阴性为:CK7,CK8,CK18,CK(H),CD34,CD117,Dog-1,Actin,SMA, Caldesmon,Desmin,CD10,CD21,CD23,CD35,CD1a,CD2,CD3,CD20,CD45, ALK,CD68,CD163,CD30,CD15,HMB45,S-100.病理诊断:肝肉瘤样癌.结论:肝肉瘤样癌是一种罕见的高度恶性肿瘤,临床与病理上需要与肝癌肉瘤、炎症性滤泡树突细胞肉瘤、炎性肌纤维母细胞肉瘤等相鉴别,预后与肿瘤分期有关.     

Carney三联征临床病理分析1例

目的:探讨Carney三联征的临床病理特征、生物学行为及预后.方法:收集1例Carney三联征临床资料,光镜下观察其组织形态学特征并行免疫组织化学分析,对相关文献资料进行回顾分析与总结.结果:患者年轻女性,先后出现肺多发性软骨瘤及胃肠道间质瘤.镜下肺软骨瘤由境界清楚的软骨小叶构成,小叶间被纤维血管分隔;胃肠道间质瘤表现为胃黏膜下多发结节,镜下肿瘤细胞呈巢团状在肌壁间浸润性生长,瘤细胞呈上皮样,圆形或多角形,胞质丰富红染,显中度异型性,核分裂像易见;免疫组织化学染色肿瘤细胞CD34、CD117、Vimentin和PDGFRA阳性.结论:Carney三联征好发于年轻女性,包括肺多发性软骨瘤、胃肠道间质瘤和肾上腺外副神经节瘤,可同时出现,也可仅存在二联征.     

原发性肝脏平滑肌肉瘤诊断和治疗体会

目的探讨原发性肝脏平滑肌肉瘤（PHLMS）的诊断和鉴别诊断。方法应用组织病理学、免疫组织化学方法观察3例原发性肝脏平滑肌肉瘤的肝脏病理切片。结果组织学观察瘤细胞呈梭形，可见核分裂，免疫组织化学结果Smooth Muscle Actin（＋）、CD117（-）、S-100（-）、Vimentin（-）。结论原发性肝脏平滑肌肉瘤是一种罕见的恶性间叶肿瘤，其诊断主要依靠免疫组织化学检查。     

原发性肺胸区上皮样血管肉瘤1例报告

<正>血管肉瘤(Angiosarcoma,AS)是一种血管内皮起源的恶性软组织肿瘤，占所有肉瘤的1%～2%^[1]。上皮样血管肉瘤(epithelioid angiosarcoma,EAS)为其形态学变异，以空泡状核、核仁明显等上皮样瘤细胞排列呈脉管样、乳头状、实性片状为特点，恶性度高，侵袭能力更强，占所有AS的30%左右。胸膜及肺的AS均较为罕见[2-5]，缺乏特异性临床表现，极易被误诊。现报道1例原发性肺胸区上皮样血管肉瘤(Primary pulmonary and thoracic EAS,PPTEAS)，总结分析其临床病理学特点，并复习相关文献，旨在进一步提高对该肿瘤的认知水平。     

肺血管肉瘤的诊断与治疗——附1例报告并文献复习

目的复习肺血管肉瘤的临床表现、病理形态学特点、诊断、鉴别诊断和治疗。方法结合文献报道,回顾性分析5例肺血肉瘤患者临床资料。结果肺血管肉瘤临床症状为胸痛、咳嗽、咯血,肺内多发结节、斑片影,病理检查瘤体主要为血管内衬异型的瘤细胞（血管内皮细胞）,可见相互吻合大小不一形态不规则的血管网,免疫组化CD34、CD31和vimentin阳性。结论肺血管肉瘤是罕见的软组织高度恶性肿瘤,预后差。肺血管肉瘤需与肺腺癌、肺结核、真菌等疾病鉴别。     

肺血管肉瘤的诊断与治疗——附1例报告并文献复习

目的复习肺血管肉瘤的临床表现、病理形态学特点、诊断、鉴别诊断和治疗。方法结合文献报道,回顾性分析5例肺血肉瘤患者临床资料。结果肺血管肉瘤临床症状为胸痛、咳嗽、咯血,肺内多发结节、斑片影,病理检查瘤体主要为血管内衬异型的瘤细胞(血管内皮细胞),可见相互吻合大小不一形态不规则的血管网,免疫组化CD34、CD31和vimentin阳性。结论肺血管肉瘤是罕见的软组织高度恶性肿瘤,预后差。肺血管肉瘤需与肺腺癌、肺结核、真菌等疾病鉴别。     

Persistent hemoptysis secondary to extensive epithelioid angiosarcoma

Pulmonary angiosarcoma is an unusual cause of diffuse pulmonary hemorrhage. Angiosarcomas are rare malignant vascular tumors accounting for 1% to 2% of all sarcomas. Angiosarcomas have been detected in nearly all organs, but lung involvement is unusual, accounting for less than 7%. The literature describes approximately 10 isolated cases of primary pulmonary angiosarcoma as opposed to the more common metastatic type. Given that primary and metastatic types are clinicopathologically similar, the presence of a distant primary sarcoma must be ruled out before a diagnosis of primary pulmonary angiosarcoma can be made. A pathological diagnosis requires a finding of polygonal or oval cells with atypical irregular nuclei and vascular spaces lined with such cells surrounded by hemorrhagic phenomena. Immunohistochemical analysis is positive for specific endothelial cell markers such as CD31 and factor VIII, and coexpression of keratin is a frequent finding.     

多中心网状组织细胞增生症三例并文献复习

目的 探讨多中心网状组织细胞增生症的临床特点、诊断及鉴别诊断。方法 分析3例多中心网状组织细胞增生症的临床、X线、组织病理和免疫组织化学特征,并复习有关文献。结果 3例患者均有多发性皮肤丘疹和结节,残毁性关节炎,肌肉症状,X线片关节有侵蚀性变化,部分指骨可有囊样骨缺损;肌酶谱均正常;病理均有细胞质呈毛玻璃样的多核巨细胞;1例伴有肺部结节;2例免疫组织化学染色示CD68阳性;AE1／AE3、S－100和HHF35均阴性。结论 多中心网状组织细胞增生症是一种系统性疾病,临床上常伴有肌肉症状,易误诊为皮肌炎,应注意两者的鉴别。     

肺结节病肺泡上皮细胞CD44表达的观察

目的探讨肺结节病的肺泡Ⅱ型细胞CD44的表达与其发病机制的关系.方法对21例肺结节病、5例结核病、3例Wegener肉芽肿、4例特发性肺纤维化和8份正常肺组织行活组织检查,行肺泡上皮总数(AE1/AE3)和CD44的免疫组化染色.3个显微镜高倍视野为观察范围,以AE1/AE3标记阳性细胞即肺泡上皮总数为基数,观察肺泡上皮的CD44阳性细胞数、以及肺泡上皮总数和CD44的计数比率.结果结节病组的肺泡上皮总数为110±32,肺泡上皮CD44的计数为84±6,与正常组的肺泡上皮总数(70±17)和CD44计数(18±4)比较,差异有显著性(P<0.001).CD44阳性表达于肺泡上皮膜上,且以肺泡Ⅱ型细胞为主.结论结节病时肺泡上皮细胞增多,伴有肺泡上皮细胞膜的CD44高表达,且以Ⅱ型细胞为主,此变化在结节病的炎症反应和修复过程中起着重要的作用.     

Bronchoscopic diagnosis of Langerhans cell histiocytosis and lymphangioleiomyomatosis

Limited data are available regarding the role of bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBB) as diagnostic tools in pulmonary Langerhans' Cell Histiocytosis (LCH) and lymphangioleiomyomatosis (LAM). The aim of this study was to review our experience regarding the value of these two techniques in the diagnosis of these cystic lung diseases. Records of 452 patients with the presumptive diagnosis of interstitial lung disease were reviewed; 67 had a clinical-radiological diagnosis of either LCH (n?=?27) or LAM (n?=?40). Of 16 patients with LCH who underwent BAL, four specimens (25%) contained cells which had positive immunoreactivity for CD1a. Of three patients with negative BAL fluid who had TBB, only one had a positive tissue diagnosis. Ten LCH patients were diagnosed by surgical lung biopsy of which five had negative BAL fluid. The remaining 12 patients were diagnosed by clinical and radiologic features. Standard examination of BAL fluid was of no diagnostic value in LAM. TBB was performed in seven patients and was diagnostic in six, not resulting in complications. All 13 patients who underwent surgical lung biopsies had a positive histopathologic diagnosis The remaining 21 patients were diagnosed by clinical and radiologic features. We suggest that BAL may assist in the diagnosis of LCH whereas TBB may be useful in the diagnosis of LAM, thus avoiding the need for surgical biopsy.     

原发性肠淋巴瘤与克罗恩病的鉴别诊断

背景：原发性肠淋巴瘤的发病率较低，临床表现缺乏特异性，易误诊为其他胃肠道疾病，特别是与克罗恩病（CD）的鉴别诊断较困难。目的：通过分析原发性肠淋巴瘤和CD患者的临床资料，提高原发性肠淋巴瘤与CD的鉴别诊断水平。方法：回顾性分析原发性肠淋巴瘤和CD患者的临床特点、内镜表现和病理检查结果。结果：原发性肠淋巴瘤患者的平均年龄为50岁，CD为35岁。5例（12.2％）CD发生肛周病变，4例（9.8％）内瘘，3例（7.3％）有肠外表现，但仅1例（5.9％）原发性肠淋巴瘤曾有肛瘘。内镜下原发性肠淋巴瘤以回盲部受累多见（41.7％），CD以小肠和结肠均受累多见（48，8％）；原发性肠淋巴瘤以肿块型较多见（41.7％），CD则主要表现为溃疡和铺路石样改变，其中18例伴肠腔狭窄，4例瘘管形成。B细胞性原发性肠淋巴瘤13例，T细胞性4例。12例CD表现为非干酪样坏死性肉芽肿。结论：临床上原发性肠淋巴瘤与CD的鉴别诊断较困难，需综合各种检查手段，特别是内镜检查和内镜下对病变部位多点取活检，以提高诊断率。     

Highly tumorigenic lung cancer CD133+ cells display stem-like features and are spared by cisplatin treatment

The identification of lung tumor-initiating cells and associated markers may be useful for optimization of therapeutic approaches and for predictive and prognostic information in lung cancer patients. CD133, a surface glycoprotein linked to organ-specific stem cells, was described as a marker of cancer-initiating cells in different tumor types. Here, we report that a CD133⁺, epithelial-specific antigen-positive (CD133⁺ESA⁺) population is increased in primary nonsmall cell lung cancer (NSCLC) compared with normal lung tissue and has higher tumorigenic potential in SCID mice and expression of genes involved in stemness, adhesion, motility, and drug efflux than the CD133⁻ counterpart. Cisplatin treatment of lung cancer cells in vitro resulted in enrichment of CD133⁺ fraction both after acute cytotoxic exposure and in cells with stable cisplatin-resistant phenotype. Subpopulations of CD133⁺ABCG2⁺ and CD133⁺CXCR4⁺ cells were spared by in vivo cisplatin treatment of lung tumor xenografts established from primary tumors. A tendency toward shorter progression-free survival was observed in CD133⁺ NSCLC patients treated with platinum-containing regimens. Our results indicate that chemoresistant populations with highly tumorigenic and stem-like features are present in lung tumors. The molecular features of these cells may provide the rationale for more specific therapeutic targeting and the definition of predictive factors in clinical management of this lethal disease.     

Multicentric Epithelioid Angiosarcoma of the Bone

ABSTRACT: Epithelioid angiosarcoma of the bone represents a challenging diagnosis by bone marrow biopsy. We present a case of a multicentric high grade angiosarcoma of the bone with epithelioid features. On the basis of the clinical presentation, the radiological findings, and the appearance of loosely clustered tumor cells detected in the initial bone marrow biopsy, the main differential diagnoses considered were a poorly differentiated non-secretory multiple myeloma and metastatic carcinoma. Subsequent morphologic, immunohistochemical and electron microscopic examination of tissue samples clarified the nature of the tumor as epithelioid angiosarcoma. We discuss potential pitfalls in clinical and morphological diagnosis. The strong reactivity of the tumor cells with the nonspecific but ubiquitous mesenchymal marker vimentin in similar cases should direct early attention to the rare malignant bone tumor, epithelioid angiosarcoma, with subsequent confirmation of this diagnosis with specific immunohistochemical endothelial cell markers and/or electron microscopy.     

结核性脓胸并发胸膜血管肉瘤一例并文献复习与分析

目的 总结分析结核性脓胸并发胸膜血管肉瘤的临床特点,提高临床诊疗水平。方法 对2016年11月山东大学附属山东省胸科医院收治的1例结核性脓胸并发胸膜血管肉瘤患者的临床表现、实验室检查结果、治疗及预后进行分析,并复习国内外文献资料。以“pleural angiosarcoma”为检索词检索PubMed数据库;以“胸膜血管肉瘤”为检索词检索万方医学网和中国知网数据库,检索时间为1995年1月至2018年11月;收集患者的一般情况、既往病史、影像学表现、病理免疫组织化学检测结果、治疗方法、预后及病程时间。结果 患者,男,56岁。因“胸闷、咳嗽超过1个月,痰中带血20d”入院,住院期间多次行胸部手术,病理诊断为结核性脓胸并发胸膜血管肉瘤。患者发病17个月后因病死亡。通过文献复习与筛选共获得相关文献31篇,包含32例胸膜血管肉瘤患者,加上本例患者共计33例;其中男23例,女10例,年龄24~87岁,平均(64.12±13.90)岁。33例患者中有结核性脓胸病史者9例,有脓胸病史者1例;有放化疗病史者2例,单纯放疗病史者1例;有粉尘接触史者1例。病灶位于右侧胸腔14例,左侧胸腔12例,双侧胸腔7例;发病时单纯胸腔积液者4例,胸膜增厚、胸膜肿物者12例,胸腔积液并发胸膜增厚、胸膜肿物者17例;明确有血胸者16例;发生转移病灶5例;CD31阳性者27例,CD34阳性者17例,Vimentin阳性者15例,Ⅷ因子相关抗原阳性者9例。33例患者均为病理确诊。治疗方法有手术(14例)、化疗(10例)、放疗(6例)、介入动脉栓塞(2例)、微波消融(1例)、胸膜固定(3例)、血管靶向药物治疗(5例)。患者病程为2个月至15年,中位时间为7个月;最终24例患者死亡,预后不详、失访和末次随访时存活者各3例。结论 胸膜血管肉瘤临床表现缺乏特异性,结核性脓胸易导致胸膜血管肉瘤的发生,当结核性脓胸患者并发不明原因的渗血性病变时,应考虑到本病的可能,防止误诊、漏诊。     

Primary intravascular large B-cell lymphoma of the lung: a review and case report

Objective

To investigate the clinicopathological features of primary intravascular large B-cell lymphoma (IVLBCL) of the lung.

Methods

Histopathological and clinical data based on lung biopsy were analyzed and used to diagnose a patient with IVLBCL of the lung.

Results

Fever and respiratory symptoms were the main presentations, lung biopsy revealed lymphoma cells in the lumen of small blood vessels. Tumor cells expressed Bcl-2, the Bcl-6, CD20, Ki67, MUM-1, Pax5, CD, CD30, and vascular endothelial CD34.

Conclusions

Primary pulmonary IVLBCL of the lung is extremely rare, on chest CT it manifests as diffuse ground glass shadow, or nodular consolidations in the lung, lactate dehydrogenase and C-reactive protein was found to increase, fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) is an important and significant diagnostic modality in its early diagnosis. Also, bronchial lung biopsy has the advantage of less trauma and high sensitive rate. R-CHOP is the main treatment for lung primary pulmonary IVLBCL of the lung; however, its prognosis is relatively poor.