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。     

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。     

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。     

子宫静脉内平滑肌瘤病病理特征

目的分析子宫静脉内平滑肌瘤病(intravenous leiomyomatosis, IVL)的临床病理特征。方法分析33例IVL患者临床表现,对其手术标本进行常规H-E染色及免疫组织化学EnVision法染色观察。 结果患者平均年龄47岁,以盆腔肿块及月经异常为主要临床表现。术前影像学及超声检查均未提示IVL。术后标本检查肿块主体直径平均6cm,多为水肿,半透明,粘冻状,质软,略不规则,周围肌层内见肿块呈蠕虫样或串珠样组织穿梭,有19例自宫体向宫旁及阔韧带穿出。光镜下肿瘤由典型的梭形平滑肌细胞组成,细胞形态温和,异型性不明显,无坏死,核分裂象罕见,伴透明样变及黏液样变,肿瘤表面覆盖一层扁平的血管内皮细胞。随访时间为6~108个月,有1例转移至肺部,3例反复复发。免疫组化染色显示肿瘤细胞ER、PR、SMA、Caldesmon、Desmin及脉管内皮细胞CD34/CD31均阳性。结论子宫静脉内平滑肌瘤病是一种少见的子宫平滑肌肿瘤,熟悉其特征性表现,仔细检查并全面取材可减少漏诊率,正确认识这种肿瘤的特点对病理诊断及临床选择合适的手术方式极为重要。     

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).     

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     

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。     

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。     

腹膜播散性平滑肌瘤病6例临床病理分析

目的探讨腹膜播散性平滑肌瘤病(LPD)的临床病理特点。方法通过对6例LPD的临床病理分析,探讨该病的组织发生、临床表现、病理形态特点、诊断及鉴别诊断、治疗、预后等。结果肿瘤在腹腔内呈多发性结节状生长并突出于腹膜表面,显微镜下观察肿瘤的瘤细胞呈束,相互垂直交错排列,除其发生部位和生长方式不同外,其他组织学改变类似于普通平滑肌瘤。结论LPD是发生于腹腔内的良性平滑肌增生性病变,可结合发生部位、生长方式、组织形态等确诊,注意与分化好的平滑肌肉瘤相鉴别。     

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。     

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.

     

Disseminated peritoneal leiomyomatosis: a devastating sequelae of unconfined laparoscopic morcellation

There has been growing concern surrounding the use of unconfined power morcellation in laparoscopic surgeries for uterine leiomyoma due to its associated risks and long-term clinical sequelae, including parasitic leiomyomas and disseminated peritoneal leiomyomatosis (DPL). We present a case of DPL resulting from previous laparoscopic morcellation and a review of the existing literature. DPL is a potentially devastating consequence of unconfined laparoscopic morcellation in the surgical management of uterine fibroids. A multidisciplinary approach is recommended in the management of DPL, especially in cases of multivisceral involvement. Clinical caution ought to be exercised when using power morcellators; when unavoidable, confined laparoscopic morcellation offers a promising mitigation and should be adopted if practicable.     

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。     

Leiomyomatosis peritonealis disseminata associated with endocervicosis

Leiomyomatosis peritonealis disseminata （LPD） is a rare but well-documented disorder mainly found in young women. Occasionally, LPD coexists with endometriosis and/or multi-cystic mesothelioma. Endocervicosis is another uncommon condition, which was recognized as a distinct entity by Clement and Young in 1992 and has been documented involving the lymph node, pelvic peritoneum, bladder and small intestine. We described a case with a unique combination of LPD and endocervicosis.     

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。     

延伸入心脏内平滑肌瘤病的病理解剖学分型及手术治疗策略的选择

目的 探讨延伸人心脏内平滑肌瘤病(intracardiac leiomyomatosis,ICL)的病理解剖学分型方法,以利于手术策略的选择.方法 回顾性分析1995年2月至2007年3月北京安贞医院收治的13例ICL的诊治资料.按其病理解剖学特征进行4级分型,13例ICL均在全麻经胸部切口或胸腹联合切口、或腹部切口,建立体外循环下,分别在常温或中低温心脏停跳或深低温低流量或深低温停循环下切除ICL.结果 全组无手术死亡.随访5～106(49±42)个月,1例于术后5个月复发死亡,应用Kaplan-Meier生存曲线计算ICL术后5年生存率为93%±5%,余12例随访无症状.术后血管超声检查于下腔静脉、双侧肾静脉、双侧髂静脉内均未见肿瘤栓子.12例患者术后心功能I级9例,Ⅱ级3例.结论 ICL手术治疗可取得较好的中长期生存率和生存质量.ICL4级分型方案有利于对ICL临床症状的分类理解和ICL手术策略的选择.     

子宫静脉内平滑肌瘤病19例临床分析

目的 探索子宫静脉内平滑肌瘤病(IVL)的临床诊断和处理.方法 回顾性分析 2005年10月--2010年11月经手术、病理确诊子宫静脉内平滑肌瘤病19例患者的临床资料.结果 术前无一例确诊,全部患者均接受手术治疗.结论 子宫IVL术前诊断困难,术中能否正确诊断处理关系到患者预后.提高术中确诊率、彻底切除原发和转移病灶...     

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。