累及右侧心腔的静脉内平滑肌瘤病(附2例报告)

目的探讨累及右侧心腔的静脉内平滑肌瘤病的诊断和治疗方法。方法对2例累及右侧心腔的静脉内平滑肌瘤病患者采用腹部B超和心脏超声、增强CT等检查。手术治疗官分期进行,一期行心脏及下腔静脉肿瘤切除术,二期于术后1个月行子宫切除并盆腔清扫术。结果2例患者手术均获成功,1例随访3年,另1例随访4．5年,均存活。结论累及右侧心腔的静脉内平滑肌瘤病罕见,详尽的术前检查、分期手术切除以及相关学科的协作是根治本病的关键。     

累及心脏的静脉内平滑肌瘤病一期外科联合手术的疗效

目的 探讨累及心脏的静脉内平滑肌瘤病的多学科一期联合手术的治疗方法及疗效.方法 选择2008年3月至2019年3月河南省胸科医院心血管外科采用体外循环(CPB)辅助下经胸腹一期外科联合手术治疗的10例累及心脏的静脉内平滑肌瘤病患者,回顾性分析患者的临床资料,包括临床表现、年龄、手术方法、手术时间、术中出血量、病理结果、...     

子宫平滑肌瘤少见生长方式影像学表现

目的：探讨子宫平滑肌瘤少见生长方式影像学表现。方法：收集经手术病理证实寄生性平滑肌瘤6例、静脉内平滑肌瘤病3例、播散性腹膜平滑肌瘤病3例和良性转移性平滑肌瘤2例,分析这些少见生长方式子宫平滑肌瘤的影像学特征。结果：6例寄生性平滑肌瘤发生在盆腔腹膜,与子宫相分隔;6例CT平扫呈等密度,增强呈中度强化;2例MRI与臀肌比较,T1WI/T2WI呈等信号。3例静脉内平滑肌瘤病均有子宫平滑肌瘤病史。1例左肾静脉受累,2例经静脉累及到右心房,1例经右心房累及到右心室;CT示下腔静脉扩张,增强显示血管内和右心腔内充盈缺损,呈＂血管内血管＂征;2例MRI显示腔静脉扩张,流空效应消失。3例播散性腹膜平滑肌瘤病表现为腹膜多发结节（直径约1-2 cm）2例,腹膜和腹膜后多发大小不等结节和肿块1例。2例良性转移性平滑肌瘤表现肺多发转移灶,1例胸膜受累。结论：在有子宫平滑肌瘤病史的女性,影像学表现局部病变进展或转移时,应考虑到子宫平滑肌瘤的一些少见生长方式。     

静脉内平滑肌瘤延伸到下腔静脉和心脏的CT诊断

目的静脉内平滑肌瘤病延伸到下腔静脉和心脏是手术治疗面临的一个难题。我们探讨使用CT血管造影在该类疾病术前评估中的应用。方法复习从2010到2017年4例静脉内平滑肌瘤延伸到下腔静脉和心脏病例,研究其术前CT征象及与外科手术的关系。结果结合临床病史和CT征象,所有病人均得到正确诊断。其中3例肿瘤局限于下腔静脉,1例肿瘤突入右心房、右心室、肺动脉。所有病人均有多发盆腔软组织肿块,经髂静脉延伸到下腔静脉,并均见肺内转移。所有病例均经包括血管外科、妇科、心脏外科合作成功手术,未出现严重并发症与死亡。结论 CT血管造影可准确提示静脉内平滑肌瘤病的位置、大小、全部病变的延伸范围以利术前评估,在制定手术计划和获得良好预后方面有重要意义。     

子宫平滑肌瘤少见生长方式影像学表现

目的:探讨子宫平滑肌瘤少见生长方式影像学表现.方法:收集经手术病理证实寄生性平滑肌瘤6例、静脉内平滑肌瘤病3例、播散性腹膜平滑肌瘤病3例和良性转移性平滑肌瘤2例,分析这些少见生长方式子宫平滑肌瘤的影像学特征.结果:6例寄生性平滑肌瘤发生在盆腔腹膜,与子宫相分隔;6例CT平扫呈等密度,增强呈中度强化;2例MRI与臀肌比较,T1WI/T2WI呈等信号.3例静脉内平滑肌瘤病均有子宫平滑肌瘤病史.1例左肾静脉受累,2例经静脉累及到右心房,1例经右心房累及到右心室;CT示下腔静脉扩张,增强显示血管内和右心腔内充盈缺损,呈"血管内血管"征;2例MRI显示腔静脉扩张,流空效应消失.3例播散性腹膜平滑肌瘤病表现为腹膜多发结节(直径约1-2 cm)2例,腹膜和腹膜后多发大小不等结节和肿块1例.2例良性转移性平滑肌瘤表现肺多发转移灶,1例胸膜受累. 结论:在有子宫平滑肌瘤病史的女性,影像学表现局部病变进展或转移时,应考虑到子宫平滑肌瘤的一些少见生长方式.     

伴有心脏受累的静脉内平滑肌瘤病一例

静脉内平滑肌瘤病是一种特殊类型的子宫肌瘤,其生长方式类似恶性肿瘤,达下腔静脉甚至累及右心腔者非常罕见,具有潜在的致命性.本文报道1例伴有心脏受累的静脉内平滑肌瘤病患者.患者41岁,女性,因"间断右上腹疼痛,伴双下肢轻度肿胀20天"入院.既往有子宫肌瘤及子宫次全切除病史.超声心动示:右心房内可见团块,与心腔无黏连,左室射血分数60.00%.腹部CT示:下腔静脉全程、左侧髂总、髂内静脉内软组织肿物,病变卜极达右心房,增强扫描示肿物有明显强化.妇科及盆腔血管彩超可见下腔静脉下段、左侧髂总静脉内占位,左侧附件区髂内、外静脉之间可见一分叶状团块,其内见营养血管,子官次全切除术后.初步诊断:下腔静脉、右心房肿瘤.行胸腹联合心脏、下腔静脉伞程肿瘤切除,左侧髂内静脉结扎术,术后未予激素治疗,病理示肿瘤细胞呈梭形,未见核分裂相,无凝固性坏死,免疫组化SMA(+++),desmin(+),PR(+),ER(+),S100(-),CD117散在个别细胞(+),CD34(-),Ki67<50%.随访6个月未见肿瘤复发.该病临床表现无特异性,术前诊断率低,确诊主要依靠病理,鉴别心脏、下腔静脉原发肿瘤和血栓后,临床治疗主要是肿瘤彻底手术切除,术后短期未见复发.     

脉管内平滑肌瘤病1例病例报告及文献回顾

<正>脉管内平滑肌瘤病(Intravenous leiomyomatosis, IVL)是一种极为少见的肿瘤，兼具有良性的组织病理学行为及恶性的生物学行为。国内报道其发生率为0.22%～1.20%^[1],国外报道亦罕见，平均发病年龄45岁^[2]。有研究表明脉管内平滑肌瘤病为子宫肌瘤向脉管内生长或由脉管本身的平滑肌瘤组织增生后突向管腔的肿瘤^[3],累及静脉者占多数，故也称血管内平滑肌瘤病、静脉内平滑肌瘤病，少数也可累及淋巴管^[3],     

静脉内平滑肌瘤病的临床表现与影像学评估

目的 探讨静脉内平滑肌瘤病(IVL)的临床表现与影像学特点,以期提高对该病的诊断水平.方法 回顾性分析9例经手术病理证实的IVL患者的临床及影像学资料,分析其临床表现、X线、超声、CT及核磁共振成像特点.结果 9例患者中5例为首次发病,4例为复发患者.5例有子宫肌瘤病史.所有患者初发或复发病程中累及下腔静脉.7例患者病程中肿瘤通过下腔静脉进入心脏,其中6例经盆腔血管进入下腔静脉.首发症状4例表现为心慌气短,2例为下肢肿胀,2例为腹胀,1例为月经量增多.超声表现为盆腔(子宫)和(或)受累静脉及心腔内低回声占位,其内可见血流.CT表现为相应部位低密度占位,增强后不均匀强化.核磁共振成像表现为相应部位等T1稍长T2信号占位,静脉内流空信号消失.结论 静脉内平滑肌瘤病具有特定的临床病史及好发部位.影像学检查对该病的早期诊断、手术计划制定和随访评估具有重要价值.     

静脉内平滑肌瘤病累及下腔静脉六例的处理经验

目的评价静脉内平滑肌瘤病累及下腔静脉时的诊断和处理方式。方法回顾性分析了北京协和医院血管外科自2000年7月至2006年2月收治的6例累及下腔静脉的静脉内平滑肌瘤病。结果6例患者中3例行Ⅰ期,2例行Ⅱ期手术,手术均获得成功,无围手术期死亡或并发症,其中4例患者的原发肿瘤和静脉内瘤栓完整切除,1例患者因多次手术,肿瘤与盆腔粘连严重,残余少许肿瘤,但术后给予抗雌性激素治疗后,瘤体有明显缩小。所有患者术后均复发。结论静脉内平滑肌瘤病极为少见,血管外科临床医师应当对该病有足够的重视,该病累及下腔静脉时可以选择多种治疗方式,手术是最佳选择,能否完整切除瘤体是防止复发的关键。     

血管内平滑肌瘤病5例影像学表现

目的 总结血管内平滑肌瘤病的影像学特点及临床治疗经验.方法 回顾性分析我院2014年1月至2019年12月确诊的5例血管内平滑肌瘤病患者的临床表现、影像学资料、手术治疗情况及术后病理和免疫组织化学染色等资料,总结血管内平滑肌瘤病的影像学特点及临床治疗经验.结果 5例患者均为女性,年龄为46～60岁(平均51.8岁),均有子宫肌瘤病史,其中2例有子宫及双侧附件全切除术史.2例患者平滑肌瘤侵及右心房,表现为心功能受损症状,余3例分别以右下肢肿胀不适、腹部肿块、下腹胀就诊.4例患者行超声及下腔静脉CT造影检查,2例行盆腔或腹部MRI平扫+增强检查,1例行血管造影检查.5例患者均行血管平滑肌瘤切除+血管成形术.2例病灶累及右心房的患者中,1例患者行一期手术治疗,1例行二期手术,术后均恢复良好.1例患者因术后4年复发再次手术.1例患者因术中出血较多,未切除右侧髂总静脉内肿瘤,行肿瘤大部切除+髂总静脉起始端结扎术.5例患者的免疫组织化学染色均表现为平滑肌肌动蛋白(SMA)(+)、钙调理蛋白(CALP)(+)、抑癌基因P53(-)、CD31(-)、细胞增殖相关抗原Ki-67(-);仅1例患者雌激素受体(ER)、孕激素受体(PR)均为阳性,1例ER、PR均为部分阳性,1例为ER部分阳性、PR少量阳性,2例患者未见ER、PR染色相关资料.5例患者术后均定期复查,随访至2021年2月均未见复发.结论 血管内平滑肌瘤病无特异性临床表现,但影像学检查特征明确,结合临床表现及妇科病史可提高诊断准确性.     

Diagnosis of intravenous leiomyomatosis extending to heart with emphasis on magnetic resonance imaging

Background  Intravenous leiomyomatosis is a rare neoplasm, and its cardiac extension is often overlooked or misdiagnosed. The purpose of this study was to explore the imaging features of intravenous leiomyomatosis with cardiac extension, especially the value of magnetic resonance imaging in differential diagnosis.

Methods  Between July 2005 and August 2008, 4 cases of intravenous leiomyomatosis with cardiac extension were resected in Cangzhou Central Hospital. Three cases had echocardiography performed, two had post contrast scans of CT performed, and two had MRI performed. Between July 2005 and May 2010, 25 cases of histopathologically proven other kinds of tumors involving the inferior vena cava and right atrium were compared for discussion of differential diagnosis.

Results  Intravenous leiomyomatosis with cardiac extension demonstrated a hyperechoic elongated mobile mass extending from the inferior vena cava to the right atrium with or without evidence of protruding into the right ventricle on echocardiography. The lesion was enhanced heterogeneously on post contrast scans of CT and was of relatively lower density compared to the enhanced blood in the inferior vena cava and right atrium, with common iliac vein and the ipsilateral internal iliac and ovarian veins involved in some cases. The untreated uterus myoma demonstrated enlargement of the uterus with heterogeneous contrast enhancement. On MRI, the lesion looked like a luffa vegetable sponge on FIESTA coronal images and a sieve pore on T2-weighted axial images. All four tumors were removed successfully, and follow up of one to four years revealed no recurrence. The 25 cases of histopathologically proven other kinds of tumors involving inferior vena cava and right atrium had their own imaging features different from those seen on intravenous leiomyomatosis with cardiac extension. With reference to their medical history, differential diagnosis can often be made.

Conclusion  The imaging appearance of intravenous leiomyomatosis has some unique features, and the luffa vegetable sponge and sieve pore like appearance on MRI may be helpful for differential diagnosis.

     

Intravenous leiomyomatosis with right heart involvement-a report of 4 cases and literature review

Intravenous leiomyomatosis(IVL) is a rare benign neoplasm which originates from the smooth muscle cells and is usually confined to the pelvic venous system.Rarely,intracaval and intracardiac extension has been described.Death can occur as a result of intracardiac involvement.We reported 4 cases of IVL with right heart involvement(intracardiac leiomyomatosis,ICL).Three of them suffered recurrent sudden syncope,and the other one was totally asymptomatic.All of them were successfully treated through one-stage operation under extracorporeal circulation.     

肺静脉曲张3例CT诊断分析

目的探讨CT诊断肺静脉曲张的应用价值。方法结合文献,对3倒肺静脉曲张的x线、CT表现进行回顾性分析。结果3例均有心脏二尖瓣病变史。x线胸片于右下肺可见“肿物”影。CT见“肿物”影与左心房相连接,为右下肺静脉近端明显扩大,增强前后其CT值与左·,12房呈一致性改变。结论CT检查可对肺静脉曲张进行定位定性定量诊断。     

心脏内平滑肌病的临床特点及外科治疗

目的总结心脏内平滑肌瘤病（intracardiac leiomyomatosis,ICL）的临床特点及外科治疗经验。方法回顾分析1999年1月至2009年1月新乡医学院第一附属医院收治的5例心脏内平滑肌瘤病患者的临床资料。结果 5例患者均为女性,有明显心慌、胸闷、晕厥或静脉栓塞等症状。影像学检查在右房及下腔静脉发现瘤栓。其中4例有＂子宫肌瘤手术史＂,1例入院后B超检查发现＂子宫肌瘤＂。5例患者均接受手术治疗,其中1例行单纯心房内瘤体切除,半年后病变复发,再次手术后死于多器官功能衰竭。4例经胸腹联合切口行分期根治术,一期完整切除右房及下腔静脉内所有瘤体,6～9个月后由妇产科行二期手术治疗原发病灶,随访2年,心脏及下腔静脉内均无肿瘤复发,无明显临床症状。结论心脏平滑肌瘤临床发病率低,手术是最主要的治疗方法,彻底切净肿瘤是成功治疗的关键。     

肺癌侵犯左心房的MRI诊断价值探讨

作者将肺癌侵犯左心房的ＭＲＩ征象归纳为左心房的异常,左房周围脂肪异常和肺静脉干异常三点,并以此为诊断标准判断有无左房受侵,通过对３０例肺癌患者术前ＭＲＩ征象分析与术后结果对照研究,结果显示：ＭＲＩ诊断的敏感性、特异性、准确性分别为９３３％、８６７％、９００％。本文结合文献探讨了ＭＲＩ对该病的诊断价值     

Massive hepatic cyst presenting as right-sided heart failure

A 70-year-old woman presented with clinical features of right heart failure. Cardiopulmonary investigations included an echocardiogram, which showed a hepatic cyst compromising venous return and affecting right atrial filling and a CT abdomen showed a 15.5?×?11.5?cm-cystic mass involving the right hepatic lobe and compressing the right atrium. Percutaneous drainage of the cyst was performed. This led to complete resolution of symptoms but these recurred as the fluid re-accumulated. Subsequent definitive treatment with excision of the cyst was undertaken with symptomatic cure. This case is the first report of a hepatic cyst presenting as right heart due to compression of the right atrium.     

心电图及X线胸片与CT综合诊断肺源性心脏病的临床价值

目的分析肺源性心脏病（PHD）的心电图、x线胸片与CT表现，探讨综合应用的临床价值。方法收集经临床确诊的肺心病27例，均作常规心电图、X线胸片、胸部CT平扫，其中17例行动态心电图检查，15例行CT增强扫描。对其心电图、胸片、CT资料进行回顾性分析。结果本组病例心电图以肺型P波为多（15例，55．6％），其次是QRS综合波改变、孓T段及T波变化、房室传导阻滞。X线胸片：心脏二尖瓣型22例，普大型5例；右心室增大19例，右心房增大6例，左心室增大4例。均见肺动脉段突出（≥O．3cm），右下肺动脉干增粗（≥1．5cm）；肺门截断征21例。慢性支气管炎表现21例，肺气肿征象17例，2例合并支气管扩张。CT表现：大部分病例心脏增大，右心室增大或肥厚21例，右心房增大9例，左心室增大5例。右下肺动脉干增粗，外围动脉变细、扭曲、紊乱。慢性支气管炎表现23例，肺气肿15例，肺大泡11例，5例合并支气管扩张。心电图提示肺心病表现15例（55．6％），X线胸片诊断肺心病19例（70．3％），CT诊断肺心病22例（81．5％），三者综合诊断24例（88．9％）。结论心电图、X线胸片与CT对肺心病诊断都能提供一定的依据，三者综合应用可提高诊断率，临床应用价值更大。     

Diagnosis and surgical treatment of intraveneous leiomyomatosis extending into the heart: two cases report and review of the literature

Objective

To investigate the clinical characteristics, diagnosis and surgical treatment of intravenous leiomyomatosis (IVL), and outline the differences between Chinese and overseas cases.

Methods

Clinical data of two IVL cases, treated in our hospital, were analyzed retrospectively and the related literature was also reviewed. The data of preoperative diagnostic rate, surgical procedures, and postoperative recurrence between patients in China and other countries were compared.

Results

The first stage operation was performed successfully in 2 patients. However, they refused subsequent therapies, including a second stage operation to excise the remnants of the tumor, uterus, bilateral oviducts and ovaries, and anti-estrogen therapy. Both suffered from IVL recurrence, one at 6-month and the other at 9-month, and died at 16-month and 12-month respectively after the first stage surgery. Worldwide reports of 110 IVL cases were reviewed, which included 28 cases in China and 82 cases in other countries. In the majority of the Chinese patients, tumors only extended into the right atrium rather than the right ventricle (RA 22 cases vs RV 4 cases). However, among the overseas patients, the rate of extension into the right atrium was similar to that of extension into the right ventricle (RA 41 cases vs RV 38 cases). The rate of hysterectomies was not significantly different between Chinese and overseas patients (67.86% vs 55.9%, P=0.278). The rate of correct preoperative diagnosis in the Chinese patients was significantly lower than that in the overseas patients (32.14% vs 80%, P=0.000002), as well as the rate of complete excision of the tumor (22.7% vs 75.5%, P=0.000001). The proportion of patients who undergoing a single-stage or a two-stage operation was similar in Chinese and overseas patients. The recurrence rate was significantly higher in the Chinese patients than in the overseas patients (36.8% vs 9.1%, P=0.0055), and the patients with tumor recurrence were partial tumor excision patients.

Conclusion

The possibility of IVL should be considered if echocardiography in female patients demonstrates a tumor in the right heart and a mass in the inferior vena cava (IVC). Further imaging should be performed to confirm the diagnosis. The correct diagnosis and accurate preoperative delineation of tumor extension are essential for an optimal surgical outcome. The key point in IVL treatment is the complete excision of tumors (single-stage or two-stage surgical procedure).     

中心型肺癌肺静脉浸润的CT评价

ＣＴ评价中心型肺癌肺静脉侵润的价值。回顾性分析经手术的１５２例中心型肺癌术前ＣＴ扫描图像。２７例显示肺静脉侵润，并累及心包膜内，没有左心房内充盈缺损。１５例显示左右上肺静脉侵润，所有肿瘤都累及心包膜下。１２例显示左右下肺静脉侵润，７例有心包膜受累（５８％）。两者间统计学上有显著差异（Ｐ＜００５）。ＣＴ可应用于评价中心型肺癌肿瘤侵润范围。上肺静脉消失，高度提示肿瘤通过肺静脉累及心包内。相反，左右下肺静脉消失，肿瘤通过肺静脉侵润心包内的机率较小