肺上皮样血管内皮细胞瘤临床病理分析

目的分析肺上皮样血管内皮瘤(PEH)的临床病理特征、诊断、鉴别诊断、治疗及预后。方法对1例原发性PEH的临床病理和免疫组织化学特征进行回顾性分析,并结合相关文献复习。结果 CT检查示双肺多发性结节,行肺部肿物切除术。光镜下肿瘤为境界清楚的嗜酸性结节,中心可见均一的类似淀粉样变或软骨瘤的透明变性组织;瘤细胞呈上皮样,也可呈实性巢状,瘤细胞出现脂肪样空泡,有的空泡内含单个红细胞,实为新生的原始血管;细胞异型不明显,核分裂像罕见。免疫组化结果:肿瘤组织中表达CD34、CD31、Ⅷ因子、Vimentin、CK等标记物。结论 PEH是1种罕见的低度恶性肿瘤,好发于中年女性,临床表现无明显特异性,诊断该病需结合影像学及病理学相关检查。     

肺上皮样血管内皮瘤一例临床病理观察

目的 探讨肺上皮样血管内皮瘤的临床病理特征,提高对该病的认识.方法 对1例肺上皮样血管内皮瘤进行组织学观察及免疫组织化学标记,并复习相关文献.结果 镜下见瘤组织在肺内呈小结节状肾小球样增生,充满肺泡腔,局灶区域可见圆形、梭形的上皮样细胞区,胞质空泡形成.免疫组织化学标记肿瘤细胞表达CD34、CD31、Vimentin、CEA,而CK7、CK20、TTF-1、CK、间皮细胞、EMA均为阴性.结论 上皮样血管内皮瘤是一种低度恶性肿瘤,发生在肺内可形成多个小结节,应注意与上皮样血管肉瘤、肺腺癌、间皮瘤相鉴别.     

鼻腔原发性恶性黑色素瘤1例

患者 ,男 ,５６岁。右侧鼻塞、流血性涕１个月 ,于１９９７年３月５日入院。体检 :右侧鼻腔有新生物 ,表面暗紫色 ,触之易出血。临床诊断 :出血性鼻息肉。恶性肿瘤待排除。予以活检。病理检查 :肿瘤组织呈片块状、巢状排列 ,出血、坏死较明显。瘤细胞较大 ,多为多边形的上皮样 ,有些近于梭形 ,核大、空淡 ,核仁明显 ,核分裂象易见。少数瘤细胞胞浆内可见数量不等的黑色素颗粒。瘤细胞胞浆呈Ｆｏｎｔａｎａ（ ）、普鲁士蓝 ( -) ;免疫组化染色 :瘤细胞胞浆呈ＨＭＢ４５、Ｓ—１００及Ｖｉ ｍｅｎｔｉｎ阳性 ,ＥＭＡ、ＬＣＡ阴性。病理诊断 :…     

恶性间皮瘤鉴别诊断方法的探讨

９例恶性间皮瘤（ＭＭ）免疫组化ＣＥＡ染色均呈阴性，对照组３６例肺腺癌３０例阳性。Ｖｉｍｅｎｔｉｎ染色９例ＭＭ中８例阳性，对照组中５例肺腺癌均为阴性。组织化学胶质铁（ＣＩ）染色９例ＭＭ中８例阳性，再行透明质酸酶（ＨＣＩ）胶质铁染色则均呈阴性，对照组５例肺腺癌ＣＩ染色４例阳性，再行ＨＣＩ染色仍为阳性。电镜下ＭＭ瘤细胞表面可见密集成刷状或蓬发样微绒毛，且细而长，浆内见丰富张力微丝，肺腺癌则无。ＡｇＮＯＲ     

上皮样血管内皮瘤9例临床病理分析

摘 要：［目的］ 分析上皮样血管内皮瘤的临床病理学特点,以提高诊断能力。［方法］ 回顾性总结9例手术切除或粗针穿刺活检诊断为上皮样血管内皮瘤的临床病理学特征及免疫组化表型。 ［结果］ 患者男性5例,女性4例;年龄16~63岁,平均年龄42岁。肿瘤分别位于肺4例、肝脏2例、软组织2例、输尿管1例。镜下观察肿瘤细胞形成上皮样形态,胞浆丰富嗜酸性,条索状或不规则散在排列,内含红细胞。间质为丰富的透明变性、黏液或黏液软骨样基质,有时可见钙化、骨化及坏死。免疫组织化学染色显示肿瘤细胞不同程度表达Vimtenin、CD31、CD34、D2-40、Fli-1、第八因子相关抗原,均未表达TFE3。8例获随访,随访时间为1~24个月均健在。［结论］ 上皮样血管内皮瘤,可见于多种实质性器官。由于其临床表现及影像学特征不典型,故不易早期发现;更因其组织形态类似于上皮样细胞,而易误诊为癌;应结合组织学特征和血管源性免疫组化表型及分子检测避免误诊。     

骨原发性上皮样血管肉瘤3例临床病理分析

目的:探讨3例骨原发性上皮样血管肉瘤的临床病理特点、诊断和鉴别诊断。方法:对3例发生于骨的上皮样血管肉瘤进行光镜、组织化学和免疫组化观察,并结合文献讨论。结果:3例肿瘤分别发生在颅骨、肋骨和骨盆,镜下肿瘤由具有上皮样特征的瘤细胞构成,细胞体积大,胞质丰富带有细小空泡,细胞核大,呈空泡状或受挤压呈印戒样,有明显核仁,核分裂象多见。瘤细胞在骨小梁间呈弥漫片状、腺管状、乳头状或单排条索状排列,有散在完整的红细胞或红细胞碎片的背景,可见形状不规则、大小不一互相交织的血管腔,其内衬上皮样瘤细胞,有的呈乳头状生长。网织纤维染色可清晰的显示瘤性血管结构,并见网织纤维围绕瘤细胞巢分布。免疫组化染色,肿瘤细胞呈上皮性标记物CKpan、高分子量角蛋白、低分子量角蛋白、EMA、间质标记物vimentin以及血管内皮细胞标记物vWF阳性,CD34、CD31弱阳性(考虑因脱钙导致该抗原严重丢失)。结论:骨原发性血管肉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。     

大阴唇上皮样血管内皮细胞瘤1例

1 病案摘要 患者,女,27岁,因外阴肿物伴轻度疼痛一年就诊。妇科检查,右侧大阴唇内侧查见一肿块,体积5cm×3.5cm×2.5cm,质中,表面光滑,活动欠佳,轻压痛。术中所见:肿物位于皮下软组织,与周围组织粘连,剥离后完整切除。临床诊断:外阴囊肿。病理检查:扁圆形肿物1个,体积4.5cm×3cm×1.5cm,包膜不清,表面粘连,切面灰红色,质硬韧。镜下:瘤细胞以血管为中心呈离心性向周围增生。细胞圆形或多角形,胞浆丰富,淡嗜酸性,内有大小不等的空泡,有的空泡内含红细胞(细胞内原始血管腔),核圆呈泡状,核仁明显(图1);瘤细胞呈小巢状或条索状排列,分布于黏液样变性及玻璃样变性的间质中(图2);少部分细胞梭形,呈梭形细胞血管内皮瘤图像。细胞无明显异型性。特殊染色:黏液PAS阴性。免疫组织化学染色:瘤细胞波形蛋白、第八因子相关抗原、C-角蛋白均呈阳性,肌动蛋白、结蛋白阴性。病理诊断为右侧大阴唇上皮样血管内皮细胞瘤。     

肝上皮样血管内皮瘤的临床病理观察

目的 探讨肝上皮样血管内皮瘤（EH）的临床病理特点、鉴别诊断及预后。方法 观察4例肝EH的组织形态学特点并行免疫组化检测,并结合相关文献进行分析。结果 EH镜下肿瘤细胞为上皮样、梭形或多角形,呈条索状、小巢状生长,或在血管内呈乳头状生长,部分瘤细胞胞质空泡化,内含红细胞;核分裂像罕见,瘤细胞胞质丰富,嗜酸性,胞界不清,间质黏液样变或玻璃样变。免疫组化染色显示肿瘤细胞CD31、CD34、Vim阳性。结论 肝EH是一种少见的低度恶性的血管源性肿瘤,影像学易误诊,需依靠病理形态学及免疫组化确诊。     

原发性肋骨血管肉瘤的诊治探讨

目的：研究肋骨血管肉瘤的临床病理特征、治疗及转归。方法：对１例原发性肋骨血管肉瘤的组织病理学、免疫组化结果进行观察分析,结合文献进行讨论。结果：本例血管肉瘤组织学表现为肿瘤细胞呈梭形或上皮样细胞形态。免疫组化染色表达ＣＤ３１、ＣＤ３４。结论：肋骨原发性血管肉瘤极少见,结合组织学形态并联合应用多项血管内皮标记物可作出明确诊断。早期发现、局部手术切除和结合术后放化疗有助于延长患者生存期。     

乳腺增生性病变与其癌变的观察Ⅱ免疫组织化学研究

对１９９２年４月～１９９３年８月天津市肿瘤医院乳腺科收治的女性乳腺癌患者６３例，乳腺良性病变１２例，叶状囊肉瘤３例，分别采用免疫组化法进行癌胚抗原（ＣＥＡ）及ｃ－ｅｒｂＢ－２癌基因产物表达的测定。检测发现乳腺良性病变基本呈阴性着色，乳腺癌基本均为阳性或强阳性，增生性病变则多为弱阳性。少数ＨＥ染色尚未出现明显异型的重度乳头状瘤病和导管上皮不典型增生，其免疫组化ＣＥＡ或ｃ－ｅｒｂＢ－２呈阳性或强阳性着色，符合病理组织学考虑这两种病变均已处于早期癌变阶段的见解。     

Multiple organ metastases of pulmonary epithelioid haemangioendothelioma and a review of the literature

This report describes a case of pulmonary epithelioid haemangioendothelioma presented as multi-organ metastases including lung, liver and bone in a 56-year-old woman with an initial diagnosis made with thoracoscopic wedge biopsy. The diagnosis is confirmed through immunohistochemistry. This is a rare disease, with approximately 90 cases described in the English literature and approximately 10 cases reported in China. The case of PEH presented as multiple pulmonary nodules and metastasing to liver and bone is rare in the English literature. The rarity of this condition, the lack of clear standards for treatment, and the partial-to-complete spontaneous regression of EHE seen in some patients up to 15 years from initial detection makes it difficult to decide on the most appropriate treatment. This report may contribute to the data on clinical findings and natural history of this rare tumor.     

支气管肺癌影像学的临床诊断观察

目的根据支气管肺癌的影像学特征,分析探讨误诊原因,并总结经验以提高支气管肺癌影像学诊断的正确率。方法选取经病理诊断证实为支气管肺癌患者80例,对其影像资料进行回顾性分析,对比影像诊断与病理诊断结果,分析不同病理类型肺癌的影像学资料的特点,对误诊病例的误诊原因加以讨论。结果经影像学诊断为肺癌患者73例,误诊患者7例,误诊率达8.75%,误诊时间平均为3个月,误诊疾病主要为肺炎、慢性支气管炎、肺结核等。鳞癌误诊患者3例,腺癌误诊患者2例,其他误诊患者2例。结论对支气管肺癌的影像学资料进行仔细观察分析,结合病例临床特征,进行综合分析,能够提高诊断的正确率。     

Clinicopathologic characteristics of primary pleural epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare vascular-derived malignancy. Within the thorax, EHE originating in the pleura is less often reported than EHE originating in the lung. Pulmonary EHE has been referred to as a borderline or low-grade malignancy that exhibits an unpredictable course. It often is associated with a long life expectancy and occasional spontaneous remission. Because the natural history of pleural EHE is less well understood, a literature review was conducted to investigate the characteristics of the disease. Twenty-two cases of pleural EHE were identified. The disease occurred more often in men than women, appeared in the right thorax more often than the left, exhibited a virulent course, was usually refractory to systemic therapy, and was often associated with a short survival. These characteristics differ substantially from those of pulmonary EHE and suggest that the clinician should approach pleural EHE anticipating an especially aggressive malignancy which likely will not respond to current therapeutic measures.     

Molecular cytogenetic characterization of epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor whose pathological diagnosis can be difficult. In the literature two cases of EHE were found to harbor a balanced t(1;3)(p36.3;q25) translocation, suggesting a characteristic chromosomal rearrangement as cause for the development of EHE. In this study, 14 cases of EHE were investigated by interphase fluorescence in situ hybridization (FISH) directed against the translocation breakpoint 1p36.3. A subset of cases was also analyzed by comparative genomic hybridization (CGH) and image cytometry. Five out of eight cases that could be successfully analyzed by FISH harbored a chromosomal break in the 1p36.3 region. The break-apart signals were present in diploid nuclei, and less frequently also in tetraploid nuclei. In the latter, the chromosomal break was present twice, suggesting that polyploidy occurred after the chromosomal alteration. DNA cytometry confirmed that tetraploid cells were present in most examined cases with one case indicating almost equal amounts of diploid and tetraploid tumor cells. CGH revealed single chromosomal imbalances of unclear significance. We could confirm that EHE may harbor a recurrent mutation involving the 1p36.3 chromosomal region thus supporting the notion that the t(1;3)(p36.3;q25) translocation is a relevant genetic finding in this tumor entity.     

41例肝脏原发性恶性血管肿瘤的临床病理特点

目的 探讨肝脏原发性恶性血管肿瘤（PHMVT）的临床病理学及预后的特点。方法 回顾性分析1982年1月至2012年12月在我院手术切除并经病理组织学证实的41例PHMVT,对其临床表现、病理学特点及预后进行分析。结果 41例患者中,肝上皮样血管内皮瘤（EHE） 19例（46.3％）,平均瘤体直径为49cm（1.2～6.3cm）;肝血管肉瘤（PHA）14例（34.1％）,平均瘤体直径为8.3cm（3.0～14.0cm）;肝婴儿型血管内皮瘤（IHE）5例（12.2％）,平均瘤体直径为3.8cm（1.2～6.3cm）;肝恶性血管外皮瘤（MHP）3例（7.3％）,平均瘤体直径为7.8cm（2.1～13.0cm）。免疫组化显示41例肿瘤细胞均表达Vimentin、CD34或因子Ⅷ。EHE患者术后中位生存期为87个月,优于PHA的12个月（P＜0.05）;2例有完整随访资料的MHP患者分别于术后43个月死亡和术后84个月复发;IHE患者术后均无复发,预后良好。结论 PHMVT的病理类型与预后有关,PHMVT的恶性程度由高到低依次为PHA＞MHP＞EHE＞IHE。     

VEGF-VEGFR pathway seems to be the best target in hepatic epithelioid hemangioendothelioma: A case series with review of the literature

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor originating from endothelial cells. Clinical aspect of the disease covers a wide spectrum from a low-grade tumor to a fatal cancer. Most common sites of EHE are reported as lung, liver and bone. Hepatic EHE (HEHE) is a clinical form with an incidence of less than 1 person in a million. Due to rarity of the disease, there is no standard therapy established. Surgery and liver transplantation still seem to be the best approach if possible. However, most of the patients present with unresectable or metastatic disease. Many conventional chemotherapeutic agents and antiangiogenic drugs have been reported previously in the literature with inconsistent outcomes. Here we report 4 cases of HEHE, who benefit distinctly from anti-VEGF treatments in different settings. While combination of paclitaxel and bevacizumab resulted in partial response in 3 patients, one of them also achieved long-term disease stabilization with bevacizumab maintenance with no adverse event. Two of the patients had clear benefit from pazopanib during the course of disease. One patient was treated with thalidomide for 18 months with stable disease, and is still being followed without any treatment. Although targeting VEGF-VEGFR pathway seems to be the best approach in HEHE, randomized studies are urgently needed to support these findings.     

Malignant Intracranial Epithelioid Hemangioendothelioma Presumably Originating from the Lung: Case Report

OBJECTIVE AND IMPORTANCE: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that presents histological features and biological behavior of low-grade malignancy. The authors report a case of malignant intracranial EHE, in which surgical excisions and additional immuno-chemotherapy were ineffective. Emphasis is placed on the histological features of this rare tumor and its potential for malignancy. CLINICAL PRESENTATION: A 69-year old male presented with paresis of the right arm. Magnetic resonance imaging revealed intracranial multiple lesions in the right temporal and parietal and left frontal lobes. Chest radiography revealed a mass lesion occupying the lower lobe of the left lung that had been followed as old tuberculosis since it had not changed in size for as long as 5 years. INTERVENTION: We performed craniotomy and excised tumors. Histological examination demonstrated that the excised tumors had the features of EHE. Further intracranial recurrence after the excision of the tumors necessitated adjuvant treatment with interferon alpha-2b. However, the patient continued to deteriorate and died 3 months later. Postmortem study disclosed the tumor cells existing in the left lung, pleura, ribs, intercostal tissues and diaphragm. Other organs including liver, bone, and skin were free from tumor invasion, which indicated that the malignant EHE originated from the left lung and metastasized to the brain. CONCLUSION: To the authors knowledge, no case of malignant EHE that simultaneously involves the central nervous system and the lung has been previously reported. An extensive inspection for involvement of other organs is recommended after recognizing an intracranial EHE.     

乳腺和肺重复癌影像诊断联合穿刺活检的临床价值

目的：探讨乳腺和肺重复癌影像诊断联合穿刺活检的临床价值。方法6例乳腺癌患者发现肺部病变,先根据影像学资料判断肺部病变性质,再选取病灶最佳活检靶点行CT引导下肺部病变穿刺活检术,并分析穿刺病理结果及临床价值。结果6例肺部病变影像学分析均考虑原发性肺癌。一次穿刺成功率100％。6例中穿刺明确诊断恶性病变5例,其中4例免疫组化标记符合肺原发性腺癌。结论乳腺和肺重复癌影像学诊断联合肺部病变穿刺活检可以满足临床诊疗的要求。     

298例肺癌合并肺结核回顾性临床分析

  目的  分析肺癌合并肺结核的临床特点,以便早期识别肺癌合并肺结核患者。  方法  对同济大学附属上海市肺科医院2005年2月至2015年2月确诊的298例肺癌合并肺结核患者的临床表现、肺癌与肺结核病灶的关系、确诊时间、病理类型、确诊方法进行回顾性分析。  结果  肺癌合并肺结核患者以痰中带血为其常见的临床特征,CT影像以双肺上叶结节多见,部分病灶呈团块样病变;肺结核与肺癌病灶在同侧同叶37例（12.4%）,病灶在肺叶不同侧138例（46.3%）,病灶在肺叶部分重叠123例（41.3%）;已有肺结核病史后诊断肺癌者120例（40.3%）,已有肺癌病史后诊断肺结核者28例（9.4%）;2种疾病同时诊断者150例（50.3%）,平均确诊时间为10.9个月~10.5年;病理类型以非小细胞肺癌为主,均通过痰检测癌细胞和纤维支气管镜刷检获得明确诊断。  结论  肺癌合并肺结核患者具有一定的临床特点和CT影像特征,应及早明确诊断,对2种疾病进行早期同时治疗,以便获得较好的疗效。      

表现为磨玻璃样病变的肺腺癌临床特点与EGFR及K-RAS基因突变的关系

目的 探讨表现为磨玻璃样病变（GGO）的肺腺癌的临床特点与EGFR及K-RAS基因突变关系。方法 连续选择37例经胸腔镜手术及病理证实为肺腺癌的肺GGO病例,分析其临床特点,检测EGFR与K-RAS基因突变情况,及与各临床因素之间的关系。结果 34例行肺叶切除术,3例行肺楔形切除术,术后病理结果显示：16例为原位腺癌,13例为微浸润腺癌,8例为浸润性腺癌。EGFR基因突变22例,K-RAS基因突变1例;在单纯型GGO及混合型GGO病例中,EGFR与K-RAS基因突变均无明显差异;在女性、不吸烟病例中EGFR基因突变相较男性及吸烟病例差异具有统计学意义（P<0.05）。结论 EGFR基因在表现为GGO的肺腺癌病例中表现出较高的突变率,为肺GGO病例临床治疗提供了重要参考。