18例静脉内平滑肌瘤病临床病理分析

目的探讨静脉内平滑肌瘤病的临床病理特点和治疗策略。方法回顾性分析北京大学人民医院收治的18例静脉内平滑肌瘤病患者临床病理资料。结果 18例患者中,12例就诊于妇科,6例就诊于血管外科;中位数年龄为45岁,主诉以月经改变、腹痛及运动后黑朦为主。术前影像学检查如彩色超声、MRI、CT、血管造影等检查可初步提示该疾病,8例术前提示此诊断。18例患者均接受手术治疗,在血管外科就诊的患者中2例行盆腔肿物切除术,4例行下腔静脉(心房)病变切除术,妇科手术范围包括7例行全子宫双侧输卵管卵巢切除术,7例行全子宫切除术,2例既往曾行全子宫切除术的患者,术中切除子宫外病灶后切除双侧输卵管卵巢,1例行子宫肌瘤切除术。术中均尽可能切除肿瘤组织,术后经病理明确诊断。术后均未药物治疗,平均随访34.7个月,一例患者术后1年复发,再次手术切除复发病灶并切除双侧输卵管卵巢后随访无复发。结论静脉内平滑肌瘤病术前诊断较困难,影像学检查有辅助诊断价值。手术为主要治疗方式,无生育要求者建议切除全子宫及双侧附件。     

复发性子宫内膜异位症伴子宫内膜乳头状腺纤维瘤一例及文献复习

一、病例摘要患者女,41岁,因子宫及右附件切除术后9年,盆腔肿物切除术后5年,阴道不规则出血3年于2010年11月28日收入院。患者9年前因发现盆腔肿物2年,急性下腹痛3h入本院行全子宫切除+右附件切除+盆腔粘连松解术,术后病理回报:(右卵巢)成熟畸胎瘤,子宫下段浆膜面囊肿,被覆输卵管型上皮,部分囊壁陈旧出血,纤维增生,     

子宫静脉内平滑肌瘤病的诊治进展

子宫静脉内平滑肌瘤病（intravenous leiomyomatosis，IVL）是一种罕见的疾病，可沿血管生长，延伸到下腔静脉，甚至心脏。目前对其发病机制尚有争议，多数学者考虑其起源于子宫肌瘤直接侵入子宫肌层静脉并沿血管腔扩散所致。子宫IVL好发于40~50岁有生育史的女性，早期临床表现不典型，如累及下腔静脉或右心时，可出现腹水、肝脾肿大、呼吸困难、心力衰竭，严重者甚至猝死。手术是目前首选的治疗方法，但具体的手术方案尚无统一共识。该病复发率高，术后需长期严密随访。综述子宫IVL的发病机制及高危因素、临床及影像学表现、诊断与鉴别诊断、治疗、预后及随访，以期为该病的诊疗提供参考。     

子宫内膜癌手术治疗的进展

2009年FIGO修订了子宫内膜癌手术病理分期。FIGO分期已推行20年但仍有问题和不足。手术治疗存在以下争议:是否要求所有患者均行盆腔及腹主动脉旁淋巴结切除术、淋巴结切除的范围,年轻低危早期者能否保留卵巢。2010年NCCN子宫内膜癌指南推荐所有可行手术者均行盆腔、腹主动脉旁淋巴结切除。前哨淋巴结检测可用于诊断早期子宫内膜癌。     

子宫平滑肌瘤长入右心腔一例报道

子宫平滑肌瘤是女性生殖器官中最常见的良性肿瘤,进入静脉或淋巴管内称为脉管内平滑肌瘤病,局限于子宫可出现月经量增多、经期延长,肿瘤过大压迫可导致下肢肿胀、下腹部不适,累及髂静脉可引起腹痛,肿瘤向上生长累及下腔静脉、右心房、右心室、肺动脉等可出现呼吸困难、心悸、胸痛、胸闷、晕厥等,甚至可出现突发性大出血及休克。报道1例49岁女性患者因子宫平滑肌瘤长入右心腔导致下肢水肿3年,胸闷、胸痛2个月的病例,入院完善检查后予经腹全子宫+双侧附件+盆腔包块切除术,开胸行下腔静脉、右心房、右心室、肺动脉肿物清除术及房间隔缺损修补术,结合术中及病理结果,确诊为子宫平滑肌瘤、血管内平滑肌瘤。好转出院,随访8个月,肿瘤未复发。子宫平滑肌瘤侵入血管长入右心室达肺动脉者极少,病情严重,早发现、早诊断、早治疗尤为重要。     

保留盆腔植物神经的根治性子宫切除术

子宫颈癌的手术治疗源于欧洲.19世纪末,Wertheim 及Meigs开拓性的工作确立了宫颈癌的"Wertheim-Meigs"手术,即根治性子官切除+盆腔淋巴清扫术,被奉为经典,沿用至今.经过不断发展和改进,根治性子宫切除+盆腔淋巴清扫术成为早期宫颈癌患者首选的治疗方法,在宫颈癌的治疗中具有重要的地位.1974年,Piver等[1]将宫颈癌手术分为5类,其中PiverⅢ类子宫切除术,又称根治性子宫切除术,是治疗宫颈浸润癌的标准术式,该手术必须打开膀胱侧窝及直肠侧窝,根据病灶的范围切断主韧带、宫骶韧带3 cm以上,阴道壁3 cm以上;PiverⅡ类子宫切除术,又称改良的根治性子宫切除术,手术需分离输尿管隧道,并切除主韧带、宫骶韧带和阴道各2cm.     

子宫平滑肌瘤长入右心腔一例报道

子宫平滑肌瘤是女性生殖器官中最常见的良性肿瘤,进入静脉或淋巴管内称为脉管内平滑肌瘤病,局限于子宫可出现月经量增多、经期延长,肿瘤过大压迫可导致下肢肿胀、下腹部不适,累及髂静脉可引起腹痛,肿瘤向上生长累及下腔静脉、右心房、右心室、肺动脉等可出现呼吸困难、心悸、胸痛、胸闷、晕厥等,甚至可出现突发性大出血及休克。报道1例49岁女性患者因子宫平滑肌瘤长入右心腔导致下肢水肿3年,胸闷、胸痛2个月的病例,入院完善检查后予经腹全子宫+双侧附件+盆腔包块切除术,开胸行下腔静脉、右心房、右心室、肺动脉肿物清除术及房间隔缺损修补术,结合术中及病理结果,确诊为子宫平滑肌瘤、血管内平滑肌瘤。好转出院,随访8个月,肿瘤未复发。子宫平滑肌瘤侵入血管长入右心室达肺动脉者极少,病情严重,早发现、早诊断、早治疗尤为重要。     

子宫内膜癌淋巴结切除意义及指征

子宫内膜癌采用手术病理分期,然而是否对所有子宫内膜癌患者都行全面分期手术(全子宫切除术+双附件切除术+双侧盆腔淋巴结及腹主动脉旁淋巴结切除)争议广泛,尤其是对于早期子宫内膜癌患者淋巴结切除的价值值得探讨。文章回顾子宫内膜癌淋巴结切除的最新研究进展,进一步讨论淋巴结切除的意义及指征。     

宫颈癌的手术治疗及并发症的处理

1　宫颈癌的手术治疗宫颈癌的治疗方案应根据患者临床分期、年龄、全身情况、设备条件和医疗技术水平来制定。有选择性的缩小手术范围 ,达到既能根治病灶 ,又能保存部分生理功能和减少术后并发症的目的。手术治疗仅适用于 a～ a期患者 ,原位癌及 a1 期患者采用筋膜外全子宫切除 ,可不清扫盆腔淋巴结 , a2 期应行次广泛子宫切除加盆腔淋巴结清扫 , b～ a期采用广泛性全子宫切除术及盆腔淋巴结清扫术 ,卵巢正常者应予保留。筋膜外全子宫切除时一般不需要分离输尿管 ,但应切除主韧带 1cm左右 ,阴道壁切除 1～ 2 cm。次广泛全子宫切除术要求…     

腹腔镜下盆腔淋巴结切除+阴式广泛宫颈切除术治疗早期宫颈癌16例临床效果及妊娠结局的观察分析

目的:探讨年轻宫颈癌保留生育功能患者行腹腔镜下盆腔淋巴结切除+阴式广泛宫颈切除术(VRT)后的临床效果及妊娠结局。方法:选择2011年1月至2015年12月就诊于宁夏医科大学总医院行腹腔镜下盆腔淋巴结切除+VRT的早期宫颈癌患者16例(VRT组),同期30例早期宫颈癌行腹腔镜下广泛子宫切除术+盆腔淋巴结切除术(LRH)患者作为对照(LRH组),对比观察两种手术的的临床效果;随访16例患者的累积妊娠率及妊娠结局。结果:两组在年龄、临床分期、病理分化程度、淋巴血管间隙受侵、鳞癌抗原表达、手术时间、术后发热率、术后并发症以及术后复发等指标上差异无统计学意义(P0.05)。VRT组术中出血量、术后住院天数明显优于LRH组(P0.05)。16例腹腔镜下盆腔淋巴结切除+VRT术后1年妊娠12例,累积妊娠次数15次;1年足月产4例次(33.3%),流产11例次(73.3%)。结论:对年轻宫颈癌患者,腹腔镜下盆腔淋巴结切除+VRT手术可达到与广泛子宫切除术+盆腔淋巴结切除术同样临床效果,同时可保留生育功能,但需提高妊娠率及生殖预后。     

Intravenous leiomyomatosis of the uterus

We present a case of intravenous leiomyomatosis (IVL) associated leiomyoma of the uterus in a 57-year-old woman. It is clinically and morphologically suspected to be leiomyoma. IVL is a rare benign neoplasm that commonly grows into the pelvic veins and the inferior vena cava and, rarely, the right side of the heart. Histologically benign smooth-muscle tumor arises from either a uterine myoma or the wall of a uterine vessel with extension into veins. The primary treatment of IVL is hysterectomy and the excision of any extrauterine tumor, when technically feasible. Antiestrogenic therapy has been suggested as potentially useful in controlling of the unresectable tumor.     

Successful One-Stage Complete Removal of an Entire Intravenous Leiomyomatosis in the Heart, Vena Cava, and Uterus

The patient was a 61-year-old woman who had intravenous leiomyomatosis (IVL) extending into the right ventricle of the heart from the uterus. Tumors in the heart and the inferior vena cava were removed under cardiopulmonary bypass and then after terminating the cardiopulmonary bypass, tumors in the abdominal cavity including uterine leiomyoma were removed. This is the first case of extended IVL in which all tumors were successfully removed at one time. If a tumor is found in the right ventricle in a woman who had or currently has uterine leiomyoma, IVL should be suspected. Complete one-stage removal of tumors can be performed using cardiopulmonary bypass, and the present patient has shown a favorable outcome.     

Intravenous leiomyomatosis misdiagnosed with large thrombosis in inferior vena cava

ObjectiveThe aim of this report is to highlight the importance of a comprehensive preoperative evaluation in the case of intravenous leiomyomatosis.Case reportA 49-year-old women was presented with dyspnea and abdominal distension. Imaging studies revealed a large leiomyoma with intravenous leiomyomatosis from this mass to the right parauterine veins, right ovarian vein reaching the inferior vena cava. Complete resection was performed by a two-stage operation by a multidisciplinary team. Final pathology confirmed it to be intravenous leiomyomatosis and uterine leiomyomas.ConclusionIntravenous leiomyomatosis is a benign and rare disease that can be a fatal condition. Precise diagnosis and appropriate treatment are important for the best outcome. Gynecologists should consider this rare disease when a patient with a uterine tumor shows symptoms such as chest pain and dyspnea.     

GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report.

BACKGROUND: Intravenous leiomyomatosis with cardiac extension is an extremely rare disease. CASE: We recently treated a case of intravenous leiomyomatosis with extension from the inferior vena cava into the right atrium. Three operations--exploratory laparotomy, debulking of the pelvic mass and resection of the intracardiac leiomyoma--were performed. Since cells of the resected leiomyomatosis were estrogen receptor positive, we postoperatively administered GnRH agonist (leuprorelin acetate) for six months to prevent regrowth of the residual mass in the pelvis. The residual mass began to enlarge immediately after cessation of leuprorelin acetate. The same medication was readministered, and regrowth of the residual mass was completely inhibited for 15 months, until this writing. CONCLUSION: Intravenous leiomyomatosis seems to be hormone dependent, as in the case of uterine leiomyomas. In the absence of total resection, functioning ovarian tissue may remain. Therefore, long-term treatment with GnRH agonist may be useful in preventing recurrence of this disease.     

Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literature

BACKGROUND: Intravenous leiomyomatosis with intracaval and intracardiac extension has been rarely described in surgical, gynecological, and radiological literatures. Complete excision of the tumor is essential for a favorable outcome. Because of the uniqueness of this tumor having an absent or localized attachment site, its removal is feasible when assisted, prior to surgery, with appropriate imaging and planning. CASE: The case was a 46-year-old woman, with intravenous leiomyomatosis originating from the uterus and extending to the inferior vena cava and right atrium, with extensive intracaval attachment, diagnosed from the various preoperative studies and operated successfully through the single-stage approach using cardiopulmonary bypass. CONCLUSION: We present an unusual case of intravenous leiomyomatosis originating from the uterus and extending to the inferior vena cava and right atrium with extensive intracaval attachment. We include a brief review of the literatures.     

Intravascular leiomyomatosis: unusual variant of leiomyoma

Leiomyomas (fibroid) arise from the smooth muscle of the myometrium and are benign in nature. Intavascular leiomyomatosis is an unusual and rare condition where the fibroid grows into the pelvic veins and inferior vena cava and sometimes extends into the heart chambers. We present a case where a woman presented to us with multiple fibroids and underwent an abdominal hysterectomy. The diagnosis was not suspected before the surgery but diagnosed on histopathology. The patient underwent further surgery to remove the fibroids from the pelvic veins and the inferior vena cava. This condition has been reported in only few case reports around the world. In view of the rarity of this condition, the diagnosis can be easily missed. We suggest an increased vigilance in women who have large fibroids with unusual features in symptoms or during surgery. Pre-surgical imaging will help to make diagnosis, and good surgical outcomes can be achieved by a multidisciplinary surgical approach.     

Intravenous uterine leiomyomatosis with diffuse dissemination in pelvic cavity. Case report and literature review

Diffuse uterine leiomyomatosis is a rare benign entity in gynecology, with only 30 worldwide reports. Its most frequent complications are dissemination into vena cava, cardiac obstruction (right cavities and valves), and diffuse pelvic invasion. We report a case of a 40 years old female with history of pelvic mass resection. During surgery it was found a large abdominal-pelvic mass diffusely invading uterine body, and round and infundibulum-pelvic ligaments. This mass extends itself out of pelvis until aorto-abdominal bifurcation, and has gummatous, elastic, and highly bleeding consistency. Histopathologic report was intravenous leiomyomatosis with benign smooth muscular fibers infiltrating adjacent structures. Its origin was uterine tissue, but it invades, adjacently, all reproductive annexes deforming them anatomy. Its infiltrative quality confers a technical difficulty to surgeon, so he or she will need a multidisciplinary team to establish its treatment.     

Intravenous leiomyomatosis with cardiac extension

Intravenous leiomyomatosis with cardiac extension is an extremely rare uterine tumor. We report here a case of a patient with a uterine leiomyoma which extended into the right atrium through the left ovarian vein, progressing into the left renal vein along the inferior vena cava. Complete one-stage removal of the tumor was performed using cardiopulmonary bypass, and the patient has shown a favorable outcome. Successful therapy for intravenous leiomyomatosis is dependent on total surgical excision of the tumor, cessation of ovarian function and avoidance of postoperative estrogen replacement therapy.     

Intracardiac leiomyomatosis

We describe a case of intracardiac leiomyomatosis originating from the uterus, growing up in the inferior vena cava, and extending into the right ventricle. She also found to have lung metastasis. As she declined for further operative intervention, Tamoxifen was given for the control of disease. This case represented an unusual rapid recurrence of intravenous leiomyomatosis which was potentially preventable. To investigate for the appropriate management, a review of the literature concerning this rare condition was made. Received: 5 June 2000 / Accepted: 26 July 2000