不同手术方式治疗延伸至心脏的静脉内平滑肌瘤病

目的:探讨延伸至心脏的静脉内平滑肌瘤病发病机制、临床特点、手术治疗方式;提高对延伸至心脏的静脉内平滑肌瘤病的认识.方法:回顾性分析兰州大学第一医院收治的3例延伸至心脏内的静脉内平滑肌瘤病患者临床资料、诊治过程,并复习相关文献.结果:3例患者均成功行手术治疗,其中2例一期一站式经胸腹联合切口行"子宫、附件、盆腔肿物切除+心脏、下腔静脉肿物取出术",另1例患者手术分2期进行,一期先行"右心房肿物切除+三尖瓣成形术",术后1周二期行"全子宫切除+双侧附件切除+下腔静脉肿物取出+右侧输尿管支架植入术".3例患者均手术成功,术后康复出院.结论:延伸至心脏的静脉内平滑肌瘤病临床少见,彻底切除瘤体是治疗本病的关键.手术需多学科联合,可一期一站式完成,也可分期分站式完成,2种手术方式临床治疗效果满意.     

MSCT对侵犯心血管系统的子宫静脉内平滑肌瘤病诊断的价值探讨

目的探讨侵犯心血管系统的子宫静脉内平滑肌瘤病MSCT诊断价值。方法回顾性分析4例经病理证实的侵犯心血管系统的子宫静脉内平滑肌瘤病的临床特点及MSCT表现并复习相关文献。结果 4例病例CT平扫均表现为子宫及子宫旁团块影,增强扫描病灶呈明显不均匀强化,其内可见增粗、迂曲的子宫或卵巢静脉影,子宫旁病灶由下至上向髂内静脉、髂总静脉、下腔静脉、右心房(室)蔓延,形如麻绳、拐杖头,其中1例可见瘤体进入肺动脉。结论 "麻绳"征、"拐杖头征"作为侵犯心血管系统的子宫静脉内平滑肌瘤病较为特殊的影像学,结合子宫及子宫旁肿块影及临床病史,可以在术前对侵犯心血管系统的子宫静脉内平滑肌瘤病作出正确的诊断,并对术前手术评估及手术方案的选择提供重要的参考价值。     

累及右心的子宫静脉内平滑肌瘤病的影像表现及临床诊治

目的总结1例累及右心的子宫静脉内平滑肌瘤病的临床诊治经验,并结合文献资料进行讨论,以利于提高对此罕见病的诊治水平。方法患者,女性,38岁,因"发热、下腹胀痛、胸闷1周"于2010年7月入院。患者G1P0,2002年因"子宫肌瘤"行"子宫肌瘤剥除术",2003年12月因"子宫肌瘤复发"行"子宫全切除术"。因强烈要求生育,拟行代孕,患者于2010年7月在美国接受注射促卵泡药物"丽申宝注射液",每日3支共计12d(剂量不详),后因发热、腹胀、胸闷自感症状加重不能承受,即回国就诊。入院后行超声、CT、MRI等影像检查以明确诊断。结果检查提示:下腹部及盆腔巨大囊实性占位性病变,左肾积水、腹腔积液,右心房、下腔静脉、左肾静脉、双侧髂静脉瘤栓形成。予行胸腹联合手术,切除右心、下腔静脉等瘤栓,切除盆腔肿瘤,病理诊断为子宫静脉内平滑肌瘤病。结论子宫静脉内平滑肌瘤病属于少见病,累及心脏更罕见,近期发生增多可能与辅助生殖中性激素使用频次增加有关;影像学检查可辅助诊断,最终确诊依赖病理;手术切除是首选治疗方式,须切除彻底,防止复发。     

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.     

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。     

静脉内子宫平滑肌瘤病一例报告

目的 报道1例罕见的静脉内子宫平滑肌瘤病病例。方法　通过病史,体检、手术所见和病理检查结合文献,分析讨论。结果　病理诊断为子宫静脉内平滑肌瘤病伴壁间型平滑肌瘤。结论　本病是一种少见的具有侵袭性生物学行为的良性肿瘤。肿瘤可沿腔静脉生长直至右心房而产生相应症状。可复发并发生肺转移,但预后良好。病理上需与内膜间质肉瘤,平滑肌肉瘤和子宫肌瘤伴血管浸润行鉴别诊断。     

静脉内子宫平滑肌瘤病一例报告

目的 报道1例罕见的静脉内子宫平滑肌瘤病病例。方法　通过病史,体检、手术所见和病理检查结合文献,分析讨论。结果　病理诊断为子宫静脉内平滑肌瘤病伴壁间型平滑肌瘤。结论　本病是一种少见的具有侵袭性生物学行为的良性肿瘤。肿瘤可沿腔静脉生长直至右心房而产生相应症状。可复发并发生肺转移,但预后良好。病理上需与内膜间质肉瘤,平滑肌肉瘤和子宫肌瘤伴血管浸润行鉴别诊断。     

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.     

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。     

右心房静脉内平滑肌瘤2例治疗体会

静脉内平滑肌瘤病是一种少见的疾病,主要是由于变异的子宫平滑肌瘤所致.容易复发,转移.国内外多为散在的个案报道,目前经检索共报道约200例.而侵犯下腔静脉和心脏者更为罕见.现报道2例侵犯右心房及下腔静脉的病例.     

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.

     

静脉平滑肌瘤病延伸至右心房一例报道

累及右侧心腔的静脉平滑肌瘤病罕见并具有潜在致命性、激素依赖性及沿血管腔蔓延的特点,以手术治疗和内分泌治疗为基础的多学科综合治疗是最佳疗法并有望改善预后。文章报道1例累及右心房的静脉平滑肌瘤病病例,经心脏超声、胸部和腹部CT、CT血管造影、病理学检查明确诊断,予三苯氧胺联合诺雷德内分泌治疗并行分期手术;一期行心脏及下腔静脉肿瘤切除术获成功。     

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。     

超声心动图诊断房间隔瘤

房间隔瘤的诊断标准:房间隔中部(卵圆窝)呈局限性膨隆突向左心房或右心房>6mm。本组16例房间隔瘤均经超声心动图诊断,其中6例经手术证实。房间隔瘤突向右心房11例,突向左心房3例,随心动周期在两心房间摆动2例,房间隔瘤的膨出方向决定于两心房间的压力阶差。     

右心房条索的大体解剖

目的了解右心房内条索的形态特点及分布规律，为心脏临床提供解剖学资料。方法２２例成人尸体心脏，切开右心房，肉眼观察房腔内各种条索的形态和分布，并用米尺对部分条索进行测量。结果界嵴长４．４１ｃｍ，宽０．４８ｃｍ，梳状肌起点９．５５条，止点８．３６条。起止之间相互交织。梳状肌有部分呈游离条索。提出了门索的概念。门索远侧为丛状条索，近侧为界嵴和梳状肌。结论右心房的条索分为纵向、横向和丛状三类。门索可能具有提缩房腔和防止心房过度扩张的作用，手术应予保护。     

乙酰胆碱对房颤复杂碎裂电位的影响

目的评估乙酰胆碱对房颤犬心房复杂碎裂电位的影响。方法成年杂种犬10只随机分为AB两组。暴露左右房,多级电极导管置于左、右心房记录碎裂电位,A组于肺静脉脂肪垫处注射乙酰胆碱,B组于上腔静脉处注射乙酰胆碱。分别于右心耳（A组）、左心耳（B组）给予BURST刺激,诱发房颤观察房颤发作后碎裂电位于两组记录位点有无差异。结果 A组房颤诱发后,左房均可记录到碎裂电位,右房未能记录到碎裂电位;B组房颤诱发后,右房能记录到碎裂电位,左房未能记录到碎裂电位。结论碎裂电位产生与局部乙酰胆碱相关。     

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.     

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).     

Intra–atrial tumour thrombus secondary to hepatocellular carcinoma

Hepatocellular carcinoma (HCC) is one of the leading causes of cancer-related mortality. Hepatitis B & C accounts for most HCC occurrences. It is very rare to see a tumour thrombus extending into the right atrium of the heart as a result of the invasion of HCC. This complication has a very poor prognosis as mean survival time is about three to four months. We encountered such a rare case of hepatocellular carcinoma having extensive tumour thrombus extending into the right atrium without any cardio respiratory distress or clinical finding suggestive of cardiovascular involvement.     

胎儿心脏右心房主要结构增龄变化观测

目的观测不同胎龄心脏右心房结构、大小及变化规律。方法40例胎心标本按胎龄分为4组,对右心房内主要结构进行观测比较,用统计学方法处理数据。结果各结构总体均数(cm)如下:上腔静脉内径0.42±0.17cm、冠状窦口直径0.21±0.08cm、右房室口直径0.75±0.27cm、右房壁厚度0.08±0.03cm、卵圆孔高度和宽度0.18±0.79cm和0.32±0.11cm、第一房室间隔高度和宽度0.52±0.21cm和0.21±0.08cm。下腔静脉瓣出现率为100%,Chair′s网占7.5%。冠状窦口瓣膜出现率为97.6%。筛状瓣膜占冠状窦口瓣膜的5%。结论上腔静脉内径、右房室口直径、第一房间隔高度和宽度随胎龄的增长而增加。冠状窦口直径、右房壁厚度、卵圆孔高度和宽度变化不规则。