子宫静脉内平滑肌瘤病临床病理分析

目的:探讨子宫静脉内平滑肌瘤病(IVL)的临床病理特点。方法:回顾6例IVL患者的临床病理资料,应用免疫组化方法检测子宫IVL瘤细胞SMA、CD34、ER和PR等的表达情况。结果:6例术后病理确诊为静脉内平滑肌瘤病。结论:IVL是具有恶性生物学行为的良性病变,术前诊断率低,确诊主要依靠病理检查,治疗以手术为主。提高术中确诊率、彻底清除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临床治疗—单中心病例总结

目的探讨来源于子宫静脉内平滑肌瘤病的临床治疗情况。方法回顾性分析2012年9月—2018年9月深圳市人民医院收治的8例经病理确诊的子宫静脉起源的平滑肌瘤病患者的临床资料。结果患者平均年龄(43.14±5.28)岁;8例患者均(100%)有生育史;1例患者有子宫切除手术史;6例(75.0%)患者就诊时无临床症状,在超声检查或妇科检查时发现;1例患者因胸闷、气促、双下肢浮肿由外院转至我院心外科,后由CT明确诊断。术前无确诊病例,所有患者均由术后病理诊断。手术方式:1例行腹腔镜下全子宫+双附件切除术,1例行经腹全子宫+双附件切除术,2例行全子宫+单侧附件切除术,1例行全子宫+双附件切除+盆腔肿物切除+子宫静脉内肿物切除,1例子宫切除术后患者行子宫静脉肿物切除,1例体外循环下行右心房+下腔静脉血管平滑肌瘤切除。术后1例失访,随访的7例患者均未出现复发。结论子宫静脉内平滑肌瘤病发病率低,诊断主要依靠病理检查;手术治疗是最有效手段。     

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...     

静脉内平滑肌瘤病25例临床分析

目的探讨静脉内平滑肌瘤病的临床表现、诊断、鉴别诊断及治疗。方法回顾性分析2008年1月—2012年1月收治的25例经病理确诊的静脉内平滑肌瘤病患者的临床资料。结果患者临床表现主要为经量增多、经期缩短、腹部包块、下腹痛,25例均行手术治疗,包括子宫肌瘤挖除术3例、全子宫切除术10例、全子宫及双侧附件切除术12例,术后随访均未见复发。结论静脉内平滑肌瘤病临床表现无明显特异性,发病隐匿,手术治疗可获良效。     

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。     

6例子宫血管平滑肌瘤病的临床分析

目的 探讨子宫血管平滑肌瘤病的临床诊治.方法 回顾性分析6例子宫血管平滑肌瘤的临床病理资料.结果 患者平均年龄40.1岁;平均孕产次分别为3,1.7;33.3％患者有子宫相关手术史.临床表现主要为盆腔包块(4/6),月经改变(2/6),无1例术前确诊,均以“子宫肌瘤”收治.术中瘤体呈白色条索状分布,沿子宫血管方向生长,6例均为术后诊断.40岁以上病变弥漫者行全子宫切除及盆腔肿瘤切除,病灶局限于宫体的年轻患者行病灶切除.随访时间1-25月,1例复发(首次手术时未全部切除肿瘤).结论 子宫血管平滑肌瘤育龄期女性多见,临床表现无特异性,确诊依靠病理组织检查,手术宜彻底切除肿瘤累及区域,术后应严密随访.     

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。     

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。     

子宫静脉内平滑肌瘤病2例报告

对2例少见的子宫静脉内平滑肌瘤病(IVL)病例进行报道并探讨临床特点和诊治方法。2例患者均一期手术成功切除肿瘤,1例术前明确诊断,另1例术中偶然发现右卵巢内蚯蚓样肿瘤。IVL易被临床忽视,应注重术前检查及术中探查,根治IVL依靠彻底性手术。本病易复发,建议术后长期随访。     

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。     

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。     

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.     

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).     

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。     

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.