Intravenous leiomyomatosis(IVL),showing unusual growth patterns of uterine leiomyoma,is a rare neoplasm characterized by intravascular proliferation of a histologically benign-looking smooth muscle cell tumor mass,but...Intravenous leiomyomatosis(IVL),showing unusual growth patterns of uterine leiomyoma,is a rare neoplasm characterized by intravascular proliferation of a histologically benign-looking smooth muscle cell tumor mass,but not invading the tissue.To date,less than 300 cases have been reported and fewer than 100 cases with cardiac involvement.Imaging characteristics of IVL are still not clear so it is usually misdiagnosed before surgery.A 36-year-old woman,who had undergone hysterectomy due to hysteromyoma,presented with shortness of breath after activities.Imaging showed IVL with mass involvement of the left ovarian vein,left renal vein,left external and common iliac vein,as well as within the inferior vena cava(IVC),extending into the right atrium.The operation demonstrated that the mass had no stalk and had welldemarcated borders with the wall of the right atrium and IVC.The patient underwent a one-stage combined multidisciplinary thoraco-abdominal operation under general anesthetic.Subsequently,the pathology report confirmed IVL.IVL should be considered in a female patient presenting with an extensive mass in the right side of the heart.Imaging technology,such as echocardiogram,contrast-enhanced computed tomography and magnetic resonance imaging,can provide important information to reveal the mass,the range and path of the lesion,and relates to the surgical plan decision.Consequently,perfect and exact image examination is very necessary pre-operation.展开更多
BACKGROUND Intravenous leiomyomatosis(IVL) is a rare and complicated disease, which requires surgery by a multidisciplinary team. However, the optimal surgical approach has not been determined.CASE SUMMARY Here we rep...BACKGROUND Intravenous leiomyomatosis(IVL) is a rare and complicated disease, which requires surgery by a multidisciplinary team. However, the optimal surgical approach has not been determined.CASE SUMMARY Here we report three cases of IVL treated with different surgical approaches. All patients presented with circulation symptoms. Two patients had lower extremity edema and the other had cardiopalmus. The diagnosis of IVL was confirmed based on the imagining examinations and pathological findings. All patients underwent surgical treatment and were discharged without any complications.CONCLUSION Preoperative examination is crucial for surgical planning and surgical approach is dependent on the patient's condition and tumor involvement.展开更多
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 descr...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.展开更多
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 ...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 fight 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 asthe rate of complete excision of the tumor （22.7@0 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）.展开更多
目的观察和分析子宫静脉内平滑肌瘤病(intravenous leiomyomatosis of the uterus,IVL)的临床和病理学特征,期望更好地了解其生物学行为,为临床和病理诊断提供帮助。方法回顾分析29例IVL的临床和病理资料,采用LABC法免疫组化检测desmin...目的观察和分析子宫静脉内平滑肌瘤病(intravenous leiomyomatosis of the uterus,IVL)的临床和病理学特征,期望更好地了解其生物学行为,为临床和病理诊断提供帮助。方法回顾分析29例IVL的临床和病理资料,采用LABC法免疫组化检测desmin、SMA、CD10、vimentin、CD34、ER、PR表达。结果所有患者术前均诊断为子宫平滑肌瘤或子宫腺肌病,无1例怀疑为静脉内平滑肌瘤病。手术中,29例均行冷冻切片检查且均正确诊断为子宫静脉内平滑肌瘤病;所有病例术后经石蜡包埋病理切片检查确诊。28例全子宫切除标本中,肿块均沿脉管腔穿出宫旁或浆膜下。获访的18例患者中,1例患者在肌瘤剥出术后两次复发,行全子宫+双侧附件切除术后随访至今无复发,其余患者随访无复发。免疫组化染色显示脉管内的肿瘤细胞desmin、SMA均阳性;脉管内皮细胞CD34阳性。结论正确认识这种少见肿瘤的大体和剖面特点可减少误诊率,术中冷冻切片检查有助于提高术中诊断率和正确选择处理方式;由于肿瘤有潜在的复发性,患者术后长期随访十分必要。展开更多
文摘Intravenous leiomyomatosis(IVL),showing unusual growth patterns of uterine leiomyoma,is a rare neoplasm characterized by intravascular proliferation of a histologically benign-looking smooth muscle cell tumor mass,but not invading the tissue.To date,less than 300 cases have been reported and fewer than 100 cases with cardiac involvement.Imaging characteristics of IVL are still not clear so it is usually misdiagnosed before surgery.A 36-year-old woman,who had undergone hysterectomy due to hysteromyoma,presented with shortness of breath after activities.Imaging showed IVL with mass involvement of the left ovarian vein,left renal vein,left external and common iliac vein,as well as within the inferior vena cava(IVC),extending into the right atrium.The operation demonstrated that the mass had no stalk and had welldemarcated borders with the wall of the right atrium and IVC.The patient underwent a one-stage combined multidisciplinary thoraco-abdominal operation under general anesthetic.Subsequently,the pathology report confirmed IVL.IVL should be considered in a female patient presenting with an extensive mass in the right side of the heart.Imaging technology,such as echocardiogram,contrast-enhanced computed tomography and magnetic resonance imaging,can provide important information to reveal the mass,the range and path of the lesion,and relates to the surgical plan decision.Consequently,perfect and exact image examination is very necessary pre-operation.
文摘BACKGROUND Intravenous leiomyomatosis(IVL) is a rare and complicated disease, which requires surgery by a multidisciplinary team. However, the optimal surgical approach has not been determined.CASE SUMMARY Here we report three cases of IVL treated with different surgical approaches. All patients presented with circulation symptoms. Two patients had lower extremity edema and the other had cardiopalmus. The diagnosis of IVL was confirmed based on the imagining examinations and pathological findings. All patients underwent surgical treatment and were discharged without any complications.CONCLUSION Preoperative examination is crucial for surgical planning and surgical approach is dependent on the patient's condition and tumor involvement.
文摘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.
基金supported by a grant:the"QingLan"projectthe Department of Education of Jiangsu province,No.(JX10210540 /DG216G15020)
文摘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 fight 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 asthe rate of complete excision of the tumor （22.7@0 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）.
文摘目的观察和分析子宫静脉内平滑肌瘤病(intravenous leiomyomatosis of the uterus,IVL)的临床和病理学特征,期望更好地了解其生物学行为,为临床和病理诊断提供帮助。方法回顾分析29例IVL的临床和病理资料,采用LABC法免疫组化检测desmin、SMA、CD10、vimentin、CD34、ER、PR表达。结果所有患者术前均诊断为子宫平滑肌瘤或子宫腺肌病,无1例怀疑为静脉内平滑肌瘤病。手术中,29例均行冷冻切片检查且均正确诊断为子宫静脉内平滑肌瘤病;所有病例术后经石蜡包埋病理切片检查确诊。28例全子宫切除标本中,肿块均沿脉管腔穿出宫旁或浆膜下。获访的18例患者中,1例患者在肌瘤剥出术后两次复发,行全子宫+双侧附件切除术后随访至今无复发,其余患者随访无复发。免疫组化染色显示脉管内的肿瘤细胞desmin、SMA均阳性;脉管内皮细胞CD34阳性。结论正确认识这种少见肿瘤的大体和剖面特点可减少误诊率,术中冷冻切片检查有助于提高术中诊断率和正确选择处理方式;由于肿瘤有潜在的复发性,患者术后长期随访十分必要。
