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 OF THE UTERUS WITH EARLY PREGNANCY

Intravenous leiomyomatosis is a rare uterineneoplasm, histologically characterized by nodularmasses of benign smooth muscle growing withinthe uterine and pelvic veins. The entity has ty-pical gross and microscopic features. A case of intravenous leiomyomatosisassociated with uterine leiomyoma, adenomyosisand early pregnancy was reported. The condi-tion was recognized during surgery for uterinefibroids. This is, to our knowledge, the thirdcase of intravenOUs leiomyomatosis associatedwith pregnancy so far reported.     

Intravenous leiomyomatosis complicated by Budd��Chiari syndrome

A 76-year-old woman with intravenous leiomyomatosis complicated by Budd-Chiari syndrome is described.     

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。     

子宫静脉内平滑肌瘤13例临床分析

目的:探讨子宫静脉内平滑肌瘤病(IVL)的临床特点。方法:回顾性分析1997年8月-2009年12月云南省大理州人民医院诊治的13例子宫IVL的临床和病理资料。结果:13例IVL中,7例患者主诉盆腔包块,5例经期延长、经量增多,1例腹痛;8例盆腔包块超过妊娠12周子宫大小,超声检查提示子宫肌瘤12例,盆腔实性肿瘤1例,6例子宫肌瘤血流丰富,其中2例血流呈树枝状,术前无一例确诊为IVL。全部患者均接受手术治疗:4例行全子宫+双侧附件切除术,7例行全子宫切除术,1例行盆腔肿瘤切除术。结论:子宫IVL是具有恶性肿瘤生物学特性的良性病变,提高术中确诊率、彻底切除原发和转移病灶以及术后全面随诊有利于改善预后。     

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.     

肺良性转移性平滑肌瘤4例及文献复习

Benign metastasizing leiomyoma(BML)is a rare lesion characterized by benign-appearing smooth muscle tumor most frequently occurring in     

侵犯心脏的静脉内平滑肌瘤病诊疗经验

目的 探讨侵犯心脏的静脉内平滑肌瘤病的诊断特点和治疗方法.方法 回顾性分析北京大学人民医院血管外科2002年11月至2011年2月收治的8例心脏受累的静脉内平滑肌瘤病患者临床资料、手术方案和随访情况.结果 8例患者影像学或手术证实病变起源于盆腔,下腔静脉、心脏受累,无肺栓塞.3例表现为心悸、胸闷等心脏症状,4例表现为下肢水肿、腹胀等下腔静脉回流障碍症状,1例无明显临床症状.8例患者均有子宫肌瘤病史,6例既往曾行子宫切除术.5例在体外循环下行右心、下腔静脉内肿瘤切除,盆腔受累静脉结扎术;2例行非体外循环下腔静脉、盆腔肿瘤切除术;姑息性激素治疗1例.手术患者中2例先期在体外循环下行右心、下腔静脉内肿瘤切除术,3个月后行子宫、双附件及病变阔韧带切除术,未行激素治疗,其余5例患者接受大剂量激素治疗.超声随访15～90个月发现未行激素治疗组和激素治疗组均有2例复发.结论 侵犯心脏的静脉内平滑肌瘤病较为罕见,手术完整切除病变是治疗关键,激素治疗预防复发效果有限.

Abstract：

Objective To explore the diagnostic features and therapeutic methods of intravenous leiomyomatosis with a potential of heart invasion.Methods Eight cases of cardiac involvement with intravenous leiomyomatosis treated at our hospital from November 2002 to August 2011 were analyzed and compared. Results Either imaging or surgery confirmed that in all 8 patients the pelvic lesions originated from the inferior vena cava and heart involvement without pulmonary embolism. In 3 cases, palpitations and chest tightness were noticed. Four cases showed lower extremity edema and abdominal distension while no obvious clinical symptom was found for 1 case. All 8 cases had a history of uterine fibroids and 6 underwent previous hysterectomy. Among 8 patients, there were 5 cases of cardiopulmonary bypassing right heart, inferior vena cava tumor resection and pelvic involvement vein ligation and 2 cases of routine off-pumping of inferior vena cava with pelvic tumor resection. Palliative therapy was administered in 1 case. Two patients were resection the right heart and inferior vena cava tumor in the cardiopulmonary bypass, 3 months later were resection gynecologic uterine, double-accessories and broad ligament and were not treated with hormone.After operation, another 5 surgery patients received high-dose hormone treatments. 2 cases of recurrence in each group were found by ultrasound follow-ups from 15-90 months. Conclusion Invasion of the heart intravenous leiomyomatosis is a rare case, which can be tackled with a good effect by completely surgical resection and a limited effect on hormone therapy to prevent recurrence.     

子宫静脉内平滑肌瘤病2例及文献复习

目的 报道2例子宫静脉内平滑肌瘤，并探讨临床特点和诊治方法。方法 分析2例临床资料，并复习相关文献。结果 2例均一期成功切除肿瘤，1例术前明确诊断，另一例术中偶然发现右卵巢内蚯蚓样肿瘤。结论 本病易被忽视．应注重术前检查及术中探查，依靠彻底性手术治疗；本病易复发，建议术后长期随访。     

子宫静脉内平滑肌瘤病3例分析并文献复习

目的探究子宫静脉内平滑肌瘤病的诊断及治疗方法。方法回顾性分析解放军总医院2004年1月-2010年11月收治的3例子宫静脉内平滑肌瘤病患者的临床及病理资料,并对相关文献进行复习。结果 3例中1例月经量增多,经期延长,1例有子宫肌瘤剔除手术史,术后随访发现盆腔包块;1例无临床表现,体检发现。3例术前超声检查2例提示平滑肌瘤,1例不除外恶性可能,无一确诊,均行全子宫切除术,术后病理确诊为静脉内平滑肌瘤病。1例术后5d发现瘤体残留,另2例分别随访17和34个月未发现复发。结论子宫静脉内平滑肌瘤病是具有恶性肿瘤生物学特性的良性病变,术前诊断率低,确诊主要依靠病理检查,治疗首选手术切除,术后应长期密切随诊。     

子宫静脉内平滑肌瘤病临床病理观察

本文报告了3例子宫静脉内平滑肌瘤病,系子宫平滑肌瘤的一罕见类型,临床经过良性。病理特征为平滑肌组织呈蠕虫样伸入子宫或盆腔静脉内,瘤细胞分化良好,核分裂象少或无,瘤组织富于厚壁小血管。诊断时应与淋巴管内间质肌病、子宫平滑肌肉瘤、腹膜播散性平滑肌瘤病及良性转移性平滑肌瘤鉴别。     

子宫静脉内平滑肌瘤病2例及文献复习

目的:探讨子宫静脉内平滑肌瘤病(IVL)的临床表现和诊治要点。方法:对2例确诊IVL患者的临床资料进行回顾分析,并对相关文献进行复习。结果:2例IVL患者临床表现与子宫肌瘤的临床表现相同,术前无特异性诊断方法,病理检查是诊断该病的依据。结论:IVL无特异性临床表现,术前不易诊断,应注重术中探查及病理检查。     

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.     

子宫静脉内平滑肌瘤病3例报告并文献复习

目的：探讨子宫静脉内平滑肌瘤病（IVL）的临床特征和处理方法,提高对该病的认识。方法对已确诊的3例子宫静脉内平滑肌瘤病患者的临床资料进行回顾分析,并复习相关文献。结果子宫IVL患者与子宫肌瘤的临床表现相同,表现为经量增多、经期延长、盆腹腔包块。术前无特异性的诊断方法,术中发现肌壁间静脉内可见蜂窝状、蠕虫状结节,延伸至宫壁。宫旁静脉增粗,静脉内可见索条状组织,质韧如橡皮样。病理检查是确诊的依据。本病典型病理改变为静脉内见蠕虫样、条索状瘤体,可抽出,镜下呈良性平滑肌瘤的形态,不侵袭静脉壁。结论子宫IVL是具有恶性生物学行为的良性疾病,术前诊断率极低,术中能否正确诊断、处理关系到患者预后,需注重术中探查,提高术中确诊率。     

Leiomyomatosis of mesenteric lymph nodes associated with duodenal adenocarcinoma

Leiomyomatosis of lymph nodes is an extremely rare disease. Only a few cases have previously been reported in pelvic lymph nodes. They were related to a benign uterine leiomyoma, a metastasizing uterine leiomyoma, an endometrial adenocarcinoma, and an ovarian endometrioid carcinoma. We report on a case of leiomyomatosis of the mesenteric lymph nodes associated with a duodenal adenocarcinoma with no history of uterine leiomyoma or any gynecological malignancy. The patient, a 56-year-old woman, was found to have an adenocarcinoma of the duodenum. All mesenteric lymph nodes removed showed leiomyomatosis, which was verified by immunohistochemical study showing positive immunostaining for smooth muscle actin, desmin, and vimentin, but negative staining for HMB-45. It is necessary to make a differential diagnosis from other examples of spindle cell proliferation involving lymph nodes such as a hemorrhagic spindle cell tumor with amianthoid fibers (palisade myofibroblastoma), angiomyolipoma, lymphangiomyomatosis, inflammatory pseudotumor, and Kaposi's sarcoma.     

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。     

累及右心的下腔静脉瘤栓手术的麻醉处理

下腔静脉瘤栓主要来源于静脉内平滑肌瘤病和肝肾肿瘤。瘤体向上蔓延可累及右心、甚至肺动脉,造成严重循环梗阻。体外循环支持下的瘤体摘除术是治疗累及右心的下腔静脉瘤栓的最为有效方法。由于这种累及右心的下腔静脉瘤栓极为罕见,文献报道也多局限于手术处理的个例介绍,目前尚无临床研究或者文献综述来讨论这种累及右心的下腔静脉瘤栓手术的麻醉处理,即便是有关麻醉处理的个案报道也极为罕见。本文将在简述这类疾病的行为特点和手术方式的基础上,结合笔者在这类手术上的初步经验,探讨如何对这类手术实施麻醉和术中管理。     

子宫平滑肌瘤病的临床分析及影像学特点

目的深入分析子宫平滑肌瘤病的临床及MRI特点。方法收集近2016年1月至2018年12月间在我院治疗的6例子宫静脉内平滑肌瘤病患者的临床影像学资料,分析肿瘤形态、位置、大小、信号及强化方式、DWI信号及ADC值,将研究结果与病理诊断结果进行对比。结果大体观察,所有患者均有子宫形态的改变,多数表现为子宫增大,多数病灶仅局限在子宫内。免疫组化实验显示平滑肌分化标志物全部呈现阳性,多数激素受体、血管源性标记物阳性。6例均有病灶蠕虫样改变,均发现病灶内流空信号,仅病例3的影像学诊断与病理诊断相符。T1WI呈等信号3例,等高信号3例;T2WI呈高低混杂信号2例,稍高信号2例,混杂高信号1例,等高信号1例。6例肿瘤内部或肿瘤旁可见多发迂曲血管。肿块强化方式均为快进慢出,强化程度除病例2为管状强化,其余均为明显强化。DWI呈高信号2例、稍高信号4例,磁共振ADC值处于正常水平。结论子宫静脉内平滑肌瘤病的临床特异性较差,术前检查的诊断正确率较低。T1WI信号为等或等高,T2WI信号为高或稍高,肿瘤内部或肿瘤旁可见多发迂曲血管。     

子宫绒毛叶状分割性平滑肌瘤伴发静脉内平滑肌瘤病(附1例报告)

目的 探讨子宫绒毛叶状分割性平滑肌瘤伴发静脉内平滑肌瘤病的临床及病理学特征。方法 对1例子宫绒毛叶状分割性平滑肌瘤伴发静脉内平滑肌瘤病的病例进行病理学观察、免疫组织化学染色等并复习相关文献。结果 患者42岁,体检B超发现子宫肌层15cm×13cm×10cm大小占位,临床拟诊断子宫肉瘤。肉眼观肌壁内一不规则肿块,向浆膜外突起似胎盘绒毛样外观。镜下肿瘤主要有分化良好的平滑肌细胞、大小不等呈丛状分布的血管组成,周围基质水肿明显。同时在子宫肌层的静脉血管内有形态良好的平滑肌增生,未见超出血管外。手术行子宫及右侧输卵管切除,术后随访6月未复发。结论 子宫绒毛叶状分割性平滑肌瘤伴发静脉内平滑肌瘤病又称为绒毛叶状水泡状静脉内平滑肌瘤病,是一类非常罕见的平滑肌瘤,预后良好。由于奇特的大体外观容易诊断错误导致过度治疗,因此掌握此类良性平滑肌瘤的病理特征以防过度治疗。     

Benign metastasising leiomyoma after hysterectomy and bilateral salpingo-oophorectomy

Benign metastasising leiomyomatosis is a rare condition affecting women in the reproductive age-group with a history of uterine fibroids, who have undergone treatment by myomectomy or hysterectomy. It is characterised by development of multiple, indolent, smooth muscle tumours outside the uterus, most commonly in the lungs, and manifests several years after the uterine surgery. We describe the case of a young woman, who had undergone total abdominal hysterectomy and bilateral salpingo-oophorectomy for multiple fibroids and a right ovarian cyst. After 5 years of being on oestrogen replacement therapy, she was detected to have benign metastasising leiomyoma, for which an additional laparotomy was performed. At laparotomy, removal of the pelvic mass was associated with several complications. The metastatic lesions in the lung responded well to progestogens (megestrol acetate) alone as evidenced by regression of the lesions detected at follow-up after 6 months and 1 year.