脾血管肉瘤一例及文献复习

国际医学放射学杂志ＩｎｔＪＭｅｄＲａｄｉｏｌ２０18Sep 鸦41穴5雪：590-593脾硬化性血管瘤样结节性转化1例及文献复习李蓉孙浩然【摘要】目的探讨脾硬化性血管瘤样结节性转化（SANT ）的临床特点、影像表现及鉴别诊断。

方法分析1例脾SANT 病人的临床特征及CT 增强表现，并复习相关文献。

结果临床无明显症状，CT 增强检查显示脾实质内边界清楚低密度肿块，动态增强扫描呈渐进性、向心性强化，动脉期及门静脉期密度低于脾实质，延迟期呈等密度，肿块内可见多发点状致密钙化灶。

病理诊断为脾SANT 。

结论脾SANT 增强扫描呈渐进性、向心性强化具有一定的特征。

【关键词】脾；硬化性血管瘤样结节性转化；体层摄影术，X 线计算机中图分类号：R733.2；R445.3文献标志码：ASclerosing angiomatoid nodular transformation of the spleen:A case report and literature review LI Rong,SUN Haoran.Department of Radiology,Tianjin Medical University General Hospital,Tianjin 300052,China【Abstract 】ObjectiveTo discuss the clinical features,imaging findings and differential diagnosis of sclerosingangiomatoid nodular transformation (SANT)of the spleen.MethodThe clinical features and the CT enhancement pattern ofone case of splenic SANT were analyzed and the related literatures were reviewed.ResultsThere was no specific clinicalfinding.There was a well -defined solitary slightly hypodense mass within the spleen.The dynamic enhanced CT scan demonstrated progressive and centripetal enhancement patterns.The lesion showed relatively low attenuation compared with background splenic parenchyma on both arterial and portal venous phases,and it became isodense to the surrounding spleen on delayed phase.There were multiple punctate calcifications in the mass.The pathological diagnosis was SANT.Conclusion Progressive and centripetal enhancement patterns are the characteristic features of SANT on enhanced CT.【Keywords 】Spleen;Sclerosing angiomatoid nodular transformation;Tomography,X-ray computedＩｎｔＪＭｅｄＲａｄｉｏｌ，２０１8，41（5）：590-593作者单位：天津医科大学总医院医学影像科，天津300052通信作者：孙浩然，E-mail ：sunhaoran2006@ DOI:10.19300/j.2018.L6617临床探究与评析脾硬化性血管瘤样结节性转化（sclerosing an 鄄giomatoid nodular transformation,SANT ）是一种罕见的良性血管增生性病变。

脾血管肉瘤1例报道通过分析既往文献报道，结合我院收治的1例脾血管肉瘤患者的临床资料，整理出脾血管肉瘤的临床病理特点及临床诊治经验，以提高脾血管肉瘤的疗效。

由于脾血管肉瘤缺乏特异性症状，诊断主要依靠影像学检查，多数病变在影像学上酷似海绵状血管瘤，确诊主要依靠病理及免疫组化检查,提示血管分化的免疫组化检测指标，如CD34、CD31、SMA、Vim、F8、UEA-1、CDl05、VEGFR3等至少两项为阳性；提示组织细胞分化的免疫组化检测指标，如CD68、CD8和S100蛋白等至少有一项为阳性。

本病首选的治疗方法是脾脏切除，术后是否需要放化疗目前尚未有统一意见。

[Abstract] Through the analysis of the literature, combined with the clinical data of 1 case of splenic angiosarcoma patients in our hospital, sorting out the clinical pathologic characteristics and clinical experience in diagnosis and treatment of splenic angiosarcoma, in order to improve the efficacy of splenic angiosarcoma. Splenic angiosarcoma due to the lack of specific symptoms, the diagnosis mainly depends on imaging examination, the majority of lesions on imaging resembles a cavernous hemangioma, diagnosis depends on pathological and immunohistochemical examination, immunohistochemical detection indicators suggest that vascular differentiation：Such as, CD34, CD31, SMA, Vim, F8, UEA-1, CDl05, VEGFR3 at least two as positive; immune group tip cell differentiation detection index：Such asCD68, CD8 and S100 protein in at least one positive. Method for treatment of this disease is the first choice of spleen resection, postoperative need chemotherapy is not yet a unified opinion.[Key words] Primary splenic angiosarcoma; Splenic tumor; Diagnosis; Treatment脾血管肉瘤（spleen angioscarcoma，SA）又稱脾恶性血管内皮细胞瘤,1879年Langhans首次报道，是间叶细胞来源的高度恶性肿瘤，由脾血窦向内皮细胞分化而成。

脾动脉瘤的诊断与治疗(附一例报告及文献复习)胡鹏;余佳;王鹏;李晨;王卫星【摘要】目的了解脾动脉瘤(splenic artery aneurysms,SAA)的临床特点、影像学表现及鉴别诊断,探讨SAA的诊断和治疗经验.方法回顾性分析2015年4月收治的1例因上腹部腹痛不适1周而入院的脾动脉瘤病人,行腹部CT平扫+增强提示脾动脉破裂并周围包裹,后行介入治疗,并结合其临床资料进行文献复习.结果随访病人1个月内未出现动脉瘤复发及上消化道出血等严重并发症,现已恢复正常生活.结论脾动脉瘤是一种起病隐匿且发病率较低的血管疾病,多数无特异性表现,CT平扫+增强对诊断脾动脉瘤具有重要意义,而CT血管造影能可靠、无创、立体地显示脾动脉瘤以及与载瘤动脉等的相互关系,能显著提高脾动脉瘤的诊断.目前手术(包括介入手术)是治疗脾动脉瘤行之有效的方法,对于有症状的脾动脉瘤应及时进行手术治疗.%Objective To summarize the clinical characteristics of splenic artery aneurysms (SAA),imaging findings and differential diagnosis,and to discuss the diagnosis and treatment of SAA.Methods One case of SAA complained of pain in upper abdomen for about one week admitted in April,2015 was retrospectively analyzed.CT scan+enhanced prompted the splenic artery rupture and surrounding wrapping.The patient was given interventional therapy,and the literature review in com-bination with clinical data was done.Results This patient did not show any complication such upper gastrointestinal bleeding or recurrence of the SAA,and the patient has been in normal life.Conclusions The splenic aneurysm is a hidden and lower incidence of vascular disease.It has no specific perform-ance.The CT scan+enhancement have an important meaning for the diagnosis ofSAA.CT angiogra-phy can be a reliable,noninvasive,three-dimensional way for revealing the correlation of splenic aneu-rysm and its parent artery,and it can significantly improve the diagnosis of SAA.The effective treat-ment for SAA is surgical operation (including interventional treatment).The symptomatic SAA should be treated timely.【期刊名称】《腹部外科》【年(卷),期】2016(029)002【总页数】4页(P105-108)【关键词】脾动脉瘤;诊断;治疗【作者】胡鹏;余佳;王鹏;李晨;王卫星【作者单位】430060 武汉,武汉大学人民医院肝胆腔镜外科;430060 武汉,武汉大学人民医院肝胆腔镜外科;430060 武汉,武汉大学人民医院肝胆腔镜外科;430060 武汉,武汉大学人民医院肝胆腔镜外科;430060 武汉,武汉大学人民医院肝胆腔镜外科【正文语种】中文【中图分类】R657.6脾动脉瘤(splenic artery aneurysms,SAA)在临床上比较罕见，但具有因破裂引起腹腔大出血的潜在危险[1]。

²临床病例分析²脾脏硬化性血管瘤样结节性转化一例并文献复习汪兵史良会【摘要】目的探讨脾脏硬化性血管瘤样结节性转化（SANT）的诊断、鉴别诊断及治疗，提高对SANT的认识，避免误诊。

方法对1例SANT患者的临床、病理特征、免疫组化表达及术后随访情况进行分析并复习文献。

结果SANT是一种近几年报道的罕见疾病，因其临床表现及影像学检查无特异性，非常容易引起误诊，确诊需依赖其特征性的病理学形态及免疫组织化学染色。

结论SANT是一种比较罕见的良性病变，预后良好，行脾切除术可以治愈。

【关键词】脾肿瘤；硬化性血管瘤样结节性转化；诊断；治疗Sclerosing angiomatoid nodular transformation of spleen: a report of one case and literature reviewWang Bing, Shi Lianghui.Department of Gastrointestinal Surgery No.3, the First Affiliated Hospital ofWannan Medical College, Wuhu 241000, ChinaCorresponding author: Wang Bing, Email: wb19836@【Abstract】Objective To explore the diagnoses, differential diagnosis and therapy of sclerosingangiomatoid nodular transformation to help us to deepen the understanding of it. Methods The clinicalmanifestations, pathology, expression of immunohistochemistry, and the follow up of 1 case of SANT wasanalyzed, and the related literature was reviewed. Results SANT was a rare disease which was reportedrecent years. Because of lacking characteristic clinical course and radiologic features, it was oftenmisdiagnosed, histological appearance and immunohistochemical staining were essential to clarify thediagnosis. Conclusion SANT is a benign change in the spleen, which has a favourable prognosis and canbe cured by splenectomy.【Key words】Splenic neoplasms;Sclerosing angiomatoid nodular transformation;Diagnoses;Therapy脾脏硬化性血管瘤样结节性转化（sclerosing angiomatoid nodular transformation，SANT）是一种非常罕见的脾脏良性病变[1-2]，文献上报道的大多数为病案个例，而且本病的临床表现及影像学改变等都缺乏特异性，手术前常被误诊为脾脏的其他疾病。

脾脏原发性血管肉瘤1例及文献复习脾脏原发性血管肉瘤罕见且预后极差，临床虽强调早确诊、早治疗，但其临床表现缺乏特异性，易于误诊，现阶段临床确诊主要依靠手术全切脾脏后组织病理学检测。

为提升诊断效果，临床应探讨脾脏原发性血管肉瘤的一般表现并找到可靠的诊断方案。

本研究以1例手术病理诊断确诊患者为研究对象，重点探讨免疫组化技术在该疾病诊断中的价值。

该病例行影像学检测可见脾脏内多发性实性占位，CT于增强后明显强化，手术标本肉眼观察（巨检）及镜检均有一定特征，行免疫组化反应可见FⅧRag（+）、CD34（+++）、CD31（+++）、Vim（+++）、SMA（局灶+），其免疫组化特征可与上皮样血管内皮细胞瘤、脾窦岸细胞血管瘤等疾病有效区分，有助于指导临床诊断。

[Abstract] Primary splenic angiosarcoma is extremely rare and prognosis is poor，because of lacking specific clinical manifestations even clinical treatment focused on early diagnosis is easy to misdiagnosis，present diagnosis stage mainly depends on operation after total resection of spleen tissue pathology detection.In order to improve the effect of clinical diagnosis，it should discuss the general performance of primary splenic angiosarcoma and find a reliable diagnosis scheme.In this study，1 cases with pathologic diagnosis confirmed patients was selected as the research object，mainly to discuss the diagnosis value of immunohistochemical technique.Detection of visible in spleen of multiple solid space occupying this image of CT patients，obviously enhancement，macroscopic observation（giant check）and operation specimens giant examination had certain features，immunohistochemical reaction of visible FⅧRag（+），CD34（+++），CD31（+++），Vim（+++），SMA （focal+），the immunohistochemical features can effectively discriminate and epithelioid hemangioendothelioma，littoral cell angioma and other diseases，which may help to guide clinical diagnosis.[Key words] Spleen tumor；Angiosarcoma；Immunohistochemistry；Diagnosis脾脏原发性血管肉瘤极为罕见，预后极差，转移率高，威胁显著，受居民生活习惯改变影响，其发病率显著提升[1]。

·7JOURNAL OF RARE AND UNCOMMON DISEASES, JAN. 2022, Vol.29, No.1, Total No.150【第一作者】肖　妮，女，主治医师，主要研究方向：淋巴造血系统肿瘤。

E-mail：****************【通讯作者】肖　妮·短篇论著· 脾脏窦岸细胞血管瘤(littoral cell angioma，LCA)是发生于脾脏的一种罕见血管来源肿瘤，大部分学者认为其起源于脾脏红髓的窦岸细胞，属于网状内皮细胞系统。

LCA由Falk等[1]的研究于1991年最早报道。

迄今，国内外文献报道的数量不断增加，但仍然有限，查阅Pubmed及万方数据库，报道此肿瘤病例数约200余例。

本文就我院诊治的1例LCA病例进行临床病理方面的探讨和分析，并进行文献复习，旨在提高对该肿瘤的认识。

1 资料与方法1.1 一般资料 患者女性，52岁。

腹痛15d，发现脾脏肿物3d。

患者于15d前无明显诱因左上腹隐痛，呈阵发性，不向他处放射。

门诊CT示脾脏多发结节，注意除外结核或淋巴瘤可能。

腹部B超显示脾脏内多发结节，性质待定。

1.2 方法1.2.1 组织学 对手术切除的脾脏标本进行大小测量，垂直于脾脏长轴书页状切开，使用10%中性福尔马林固定，取材，常规脱水、石蜡包埋，切片，HE染色，光镜观察。

1.2.2 免疫组化 采用Envision二步法，所用抗体CD31、CD68购于DAKO公司，CD34、CD8、CD21、Factor8、Ki-67均购于中杉金桥公司，具体步骤按照说明书进行，并设有阴性和阳性对照。

2 结 果2.1 巨检 脾脏大小13cm×9cm×3.5cm，脾脏被膜光滑，书页状切开切面见多个暗红色结节，直径0.1cm~1.0cm(图1)。

2.2 镜检 脾脏内暗红色结节位于红髓内，境界清楚，由大小不一、相互吻合的血管腔构成，部分血管腔扩张成囊状，局灶可见突向血管腔的乳头状结构，乳头轴心为纤维血管间质。