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。     

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。     

胰腺上皮样血管肉瘤的临床病理分析

目的探讨胰腺上皮样血管肉瘤的临床病理学特征及鉴别诊断要点。方法在光学显微镜下对胰腺上皮样血管肉瘤进行组织学形态观察,并借助免疫组化进一步对血管肉瘤的形态进行分析。结果大体上为灰白色结节状,切面呈灰白色,质地中等,显微镜下组织学表现为肿瘤细胞呈上皮样、多边形,病理性核分裂象多见,弥漫分布,异型性明显,免疫组化显示肿瘤细胞高表达CK、CD31、CD34和Vimentin等,血管和间叶分化,肿瘤高度恶性。结论胰腺上皮样血管肉瘤具有高度侵袭性,转移迅速,预后差,在临床病理诊断中,必须首先与发生于该部位常见的恶性肿瘤如癌、转移癌、恶性黑色素瘤、其他肉瘤等鉴别。     

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9例临床病理分析

目的:探讨心脏原发性血管肉瘤的临床病理特点、诊断和鉴别诊断、治疗及预后特点。方法:对9例心脏原发性血管肉瘤进行光镜形态及免疫组化特征观察,并结合文献分析讨论。结果:9例中,男性5例,女性4例,年龄在19~62岁,平均39岁。镜下见肿瘤形态多样,局部呈大小不规则的血窦样结构,互相连通,腔内壁衬覆肿瘤性内皮细胞,细胞异型性明显,免疫组化:CD31(+),CD34(+),Fli-1(+),ERG(+),Vimentin(+)。结论:心脏原发性血管肉瘤是一种罕见的预后很差的恶性肿瘤,临床表现缺乏特异性,病理形态多样,诊断困难,借助免疫组化特异性标记有助于明确诊断。     

肺血管肉瘤的临床病理观察

目的 探讨肺原发性血管肉瘤的临床病理特点.方法 对我院收治的1例,发生于肺的血管肉瘤的临床表现、组织形态学及免疫组化进行分析,并复习相关文献.结果 患者男性,67岁,临床表现主要为咳嗽和痰中带血.光镜下上皮样或梭形肿瘤细胞形成不规则且相互吻合的血管腔隙,伴大片的出血坏死.免疫组织化学染色显示肿瘤细胞CD34、CD31和vim阳性;CK( AE1/AE3)、TTF-1和S-100阴性.结论 原发性肺血管肉瘤具有和其他部位血管肉瘤相似的组织形态学特点,免疫组织化学是其必要的辅诊手段.其临床表现无明显特异性,早期诊断困难.     

“此起彼伏”的肺内结节伴晕征合并脑内多发病灶

肺内血管肉瘤(epithelioid angiosarcoma)是一种罕见的肿瘤,因其导致出血而在肺内形成结节伴“晕征”的征象,随着出血的发生及吸收易形成“此起彼伏”的改变,影像表现特殊。同时伴有脑内转移的更为罕见,脑内的血管肉瘤同样易出血,核磁共振影像表现具有特异性。本文通过报道1例在肺部表现为多发结节伴晕征,随着多次随访出现“此起彼伏”改变,以及脑内多发转移的血管肉瘤,介绍该病的临床表现、影像学表现、鉴别诊断,分析并总结肺内出现结节伴晕征的疾病的鉴别诊断思路,为临床医生早期诊断该病提供参考。     

原发性肌源性肺肉瘤

目的 :报告我院 1981～ 1992年 ,经手术切除 ,根据光镜、电镜及免疫组化观察确诊的原发性肌源性肺肉瘤 6例。方法 :年龄 3～ 6 5岁 ,男性 4例 ,女性 2例 ,其所中包括横纹肌肉瘤 4例 ,肺平滑肌肉瘤和肺血管平滑肌肉瘤各 1例。结果 :6例肌源性肺肉瘤在光镜下各有其形态特点 ,免疫组化观察结果为 :结蛋白 (Desmin)及肌动蛋白 (Actin)染色瘤细胞全部呈阳性表达 ,肌红蛋白 (mb)染色肺横纹肌肉瘤瘤细胞呈阳性表达。肺平滑肌肉瘤及肺血管平滑肌肉瘤为阴性 ;S 10 0蛋白和细胞角蛋白 (Cytoker atin)全部为阴性。 3例横纹肌肉瘤电镜观察 :胞质内可见肌原纤维。结论 :原发性肺肉瘤的组织发生及其发病率 ,以及免疫组化染色在肺肉瘤诊断中的作用。     

肺上皮样血管内皮瘤的CT特征及临床分析

目的探讨肺上皮样血管内皮瘤(PEH)的CT影像特征及临床特点。 方法回顾性分析2014年1月至2020年11月就诊于我院经病理证实的4例PEH患者的临床及CT影像资料。 结果PEH患者4例均为男性,平均年龄45.8岁;2例无吸烟史、2例偶有吸烟史。初诊时有咳嗽、咳痰1例,右侧胸痛1例,无症状经体检发现2例。病理学免疫组化显示PEH瘤细胞血管内皮标记CD31 、CD34、Ki-67阳性4例。胸部CT影像显示右肺上叶肿块伴肺内少量结节2例,双肺散在多发小结节且主要集中于双肺基底段胸膜下、叶间裂附近及支气管血管束周围2例,病灶边缘常较清楚、形态较规则4例。PEH合并肝脏上皮样血管内皮瘤1例。 结论肺上皮样血管内皮瘤的CT表现具有一定特异性,结合临床可提示诊断该病,但最终需病理学确诊。     

肺上皮样血管内皮瘤一例并文献复习

目的 提高对肺上皮样血管内皮瘤的认识。方法 结合1例肺上皮样血管内皮瘤患的临床资料和献复习，详细分析该病的病因、临床表现、诊断、鉴别诊断、治疗及预后等。结果 该病病因不清，临床症状较少且轻微，影像学检查主要表现为两肺多发性结节影；病理学检查主要特征是单细胞原始管腔结构；免疫组织化学提示内皮细胞源性。目前尚无有效治疗措施，患预后介于良、恶性肿瘤之间。结论 肺上皮样血管内皮瘤罕见，极易误诊，应予重视。     

Bilateral massive bloody pleurisy complicated by angiosarcoma

We report a case of angiosarcoma complicated by bilateral massive bloody pleurisy (hematocrit of 7.2%) in an 83-year-old woman. An autopsy revealed hemorrhagic tumors infiltrating both the diaphragm and serosal surface of the peritoneum. Histological examination confirmed an anastomosing vascular channel pattern of the tumor cells with characteristic immunohistochemical findings for angiosarcoma, such as positive staining for vimentin, CD31, CD34, D2-40, and factor VIII-related antigen. The tumor was thought to have originated from a small vessel on the serosal surface of the duodenum. We also reviewed cases of hemothorax associated with angiosarcoma, which suggested that primary or metastatic pleural angiosarcoma should be considered a cause of spontaneous hemothorax, especially in patients with bilateral and/or intractable hemothorax.     

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。     

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.     

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.     

CT血管征在肺微小结节的诊断与鉴别诊断中的价值探讨

目的研究CT血管征对肺微小结节良恶性判定的临床意义。方法分析我院95例肺微小结节患者CT影象学资料,依据病理诊断将其分为研究组与对照组,研究组恶性病变,对照组良性病变。对比两组CT血管征诊断的敏感性差异、病变类型与CT血管征分型间的关系、不同时点动态增强CT值、PH、SPH/PPH间差异。结果研究组血管征阳性者CT平扫、动态增强30s、90s、180s、300s、480s CT值与对照组CT平扫、各时点动态增强CT值相较,均呈明显差异(P0.05)。研究组血管征阳性者动态增强CT特征性参数中,PH、SPH/PPH与对照组相较,均呈明显差异(P0.05)。结论肺微小结节CT血管征有辅助诊断意义,可以指导临床判定肺微小结节的良恶性病变。动态增强扫描可以提高CT血管征对肺微小结节良恶性的确诊率。     

Primary pulmonary angiosarcoma

Pulmonary angiosarcoma is an uncommon vascular and usually secondary tumor. Only a few primary cases of pulmonary angiosarcoma have been described. We report a case of primary pulmonary angiosarcoma in an adult man who presented with hemoptysis. Chest x-ray film and chest CT showed a right para-cardiac opacity associated with diffuse alveolar consolidation of the right basal lobe. Right inferior lobectomy has been performed. Pulmonary angiosarcoma was diagnosed on histological and immuno-histochemical studies of the operative specimen. The primitive character was retained after ruling out all other tumor localizations. The clinical outcome was rapidly fatal. This observation is added to the other rare cases published of primitive pulmonary angiosarcoma. It confirms the poor prognosis and the extremely hemorrhagic nature of this tumor. Isolated necrotic parenchymatous mass was an original radiological pattern observed in this patient.     

The clinical features of hepatic angiosarcoma: a report of four cases and a review of the English literature.

Four cases of hepatic angiosarcoma are reported with a review of 99 other cases in the English literature. Angiosarcoma of the liver is associated with chronic exposure to thorotrast, vinyl chloride, arsenicals, radium and possibly copper and with chronic idiopathic hemochromatosis. Although 40% of patients have hepatic fibrosis or cirrhosis at autopsy, the nature of the association between chronic liver disease and hepatic angiosarcoma is unknown. The clinical presentation of hepatic angiosarcoma is nonspecific with abdominal pain, weakness and weight loss common complaints and with hepatomegaly, ascites and jaundice common findings. Liver function tests are usually abnormal but there is no one liver function test or set of tests specific for the tumor. The occurrence of thrombocytopenia and disseminated intravascular coagulation is characteristic of hepatic angiosarcoma and may be related to local consumption of clotting factors and formed blood elements in the tumor. Catastrophic intraabdominal bleeding is also characteristic and occurs in one-fourth of all cases. This complication is likely related to the high incidence of clotting abnormalities and the vascular nature of the neoplasm. Selective hepatic arteriogram and open liver biopsy are the foundations of diagnostic evaluation. Percutaneous liver biopsy should be avoided. Failure to appreciate the possibility of hepatic angiosarcoma in the proper clinical setting, leading to blind percutaneous biopsy, may result in failure to make the diagnosis at the cost of significant morbidity and mortality. Survival of patients with hepatic angiosarcoma is brief; only 3% live longer than 2 years. Treatment of the tumor to date is empirical. There are probably a few patients who might benefit from radical surgery with curative intent. For all others chemotherapy is indicated. Adriamycin is active against hepatic angiosarcoma, but optimal dose and mode of administration require further investigation. Further study is also required to delineate the cause of hepatic angiosarcoma in the 60% of cases without definite epidemiologic association.     

A case of primary splenic angiosarcoma with intraperitoneal hemorrhage treated by transcatheter arterial embolization

Splenic tumors are very rare. In Japan only 42 cases of splenic angiosarcoma have been reported. We encountered a case of spontaneous rupture of a splenic angiosarcoma and liver metastasis. A 60-year-old woman who suddenly went into hemorrhagic shock presented at our hospital. Then acute spontaneously ruptured spleen and hepatic tumors were diagnosed by abdominal CT. After emergency TAE, the patient was hemodynamically stable, but died of liver failure 13 days after admission. The pathological diagnosis was primary splenic angiosarcoma with multiple organ metastasis on autopsy. Splenic angiosarcoma should be kept in mind in the differential diagnosis of splenomegaly or splenic tumor. TAE can be effective in primary hemostasis for angiosarcoma with intraperitoneal hemorrhage from multiple tumors.     

软骨肉瘤肺转移1例及文献复习

目的提高对软骨肉瘤肺转移的认识。方法结合一例软骨肉瘤肺转移患者的临床资料和文献复习,详细分析该病的病因、临床表现、诊断、鉴别诊断等。结果该病临床症状无特异性,确诊有赖于病理检查。结论软骨肉瘤肺转移罕见,极易误诊,应予重视。