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病三例报告

子宫静脉内平滑肌瘤病(intravennus leionlyomatosis IVL)是一种非常少见的子宫良性肿瘤,此肌瘤向子宫脉管内生长或由脉管本身的平滑肌组织增生后,向子宫外脉管腔扩展,除静脉外淋巴管也可受累,所以又称脉管内平滑肌瘤病。病理     

子宫静脉内平滑肌瘤病1例

1病例资料患者,马某,女,42岁,主因月经量增多6个月,发现盆腔包块8 d于2008年9月9日入院。患者平素月经规则,量中,无痛经。生育史:孕3产1,上环17年。半年前无诱因出现月经增多,约为原来的2倍,有血块,时有头晕乏力,但经期、周期同以往,无     

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。     

子宫静脉内平滑肌瘤病1例

病人,女,50岁。平素月经周期规律,近半年来自觉下腹部胀痛不适。妇科检查：盆腔偏左侧可扪及如孕8个月大小的肿物,质中。彩超检查：子宫稍增大,形态尚可,肌层回声增强,分布不均匀,内可见多个低回声区,较大的约为2.3 cm×2.0 cm,边界尚清。     

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.     

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。     

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).