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
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...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 l展开更多
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
Our knowledge of renal cell carcinoma(RCC) is rapidly expanding. For those who diagnose and treat RCC, it is important to understand the new developments. In recent years, many new renal tumors have been described and...Our knowledge of renal cell carcinoma(RCC) is rapidly expanding. For those who diagnose and treat RCC, it is important to understand the new developments. In recent years, many new renal tumors have been described and defined, and our understanding of the biology and clinical correlates of these tumors is changing. Evolving concepts in Xp11 translocation carcinoma, mucinous tubular and spindle cell carcinoma, multilocular cystic clear cell RCC, and carcinoma associated with neuroblastoma are addressed within this review. Tubulocystic carcinoma, thyroid-like follicular carcinoma of kidney, acquired cystic disease-associated RCC, and clear cell papillary RCC are also described. Finally, candidate entities, including RCC with t(6;11) translocation, hybrid oncocytoma/chromophobe RCC, hereditary leiomyomatosis and RCC syndrome, and renal angiomyoadenomatous tumor are reviewed. Knowledge of these new entities is important for diagnosis, treatment and subsequent prognosis. This review provides a targeted summary of new developments in RCC.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
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 excisi展开更多
BACKGROUND Leiomyomatosis peritonealis disseminata(LPD)is a rare condition characterized by multiple pelvic and abdominal nodules,which are composed of smoothmuscle cells.To date,no more than 200 cases have been repor...BACKGROUND Leiomyomatosis peritonealis disseminata(LPD)is a rare condition characterized by multiple pelvic and abdominal nodules,which are composed of smoothmuscle cells.To date,no more than 200 cases have been reported.The diagnosis of LPD is difficult and there are no guidelines on the treatment of LPD.Currently,surgical excision is the mainstay.However,hormone blockade therapy can be an alternative choice.CASE SUMMARY A 33-year-old female patient with abdominal discomfort and palpable abdominal masses was admitted to our hospital.She had undergone four surgeries related to uterine leiomyoma in the past 8 years.Computed tomography revealed multiple nodules scattered within the abdominal wall and peritoneal cavity.Her symptoms and the result of the core-needle biopsy were consistent with LPD.The patient refused surgery and was then treated with tamoxifen,ulipristal acetate(a selective progesterone receptor modulator),and goserelin acetate(a gonadotropin-releasing hormone agonist).Both tamoxifen and ulipristal acetate were not effective in controlling the disease progression.However,the patient achieved an excellent response when goserelin acetate was attempted with relieved syndromes and obvious shrinkage of nodules.The largest nodule showed a 25%decrease in the sum of the longest diameters from pretreatment to posttreatment.Up to now,2 years have elapsed and the patient remains asymptomatic and there is no development of further nodules.CONCLUSION Goserelin acetate is effective for the management of LPD.The long-term use of goserelin acetate is thought to be safe and effective.Hormone blockade therapy can replace repeated surgical excision in recurrent patients.展开更多
目的：由于子宫静脉内平滑肌瘤病（IVL）属于少见病例,其CT影像学表现有一定的特征性,回顾性分析IVL的多排CT特征及其临床表现,评价其对手术治疗的指导意义。方法：回顾性总结分析2005年至2013年间,通过外科手术及病理证实的9例IVL的患...目的：由于子宫静脉内平滑肌瘤病（IVL）属于少见病例,其CT影像学表现有一定的特征性,回顾性分析IVL的多排CT特征及其临床表现,评价其对手术治疗的指导意义。方法：回顾性总结分析2005年至2013年间,通过外科手术及病理证实的9例IVL的患者的临床资料,多排CT特征以及病理结果。多排CT扫描参数：管电压100~120 k V,管电流400~450 m As,层厚0.5~0.75 mm,间隔0.25~0.60 mm。监测层面放在下腔静脉右心房入口区域,手动触发开始扫描。结果：9例患者中,4例患者为子宫切除术后,1例为多发子宫肌瘤并伴子宫腺肌病。多排CT均显示蛇形的下腔静脉内充盈缺损延伸入右心房,其中1例病变延伸入右心室、肺动脉。8例病例侵犯单侧宫旁血管,1例病例侵犯双侧子宫血管,6例肿瘤沿着子宫静脉、髂静脉生长,3例病例显示病变同时波及卵巢静脉和髂静脉,9例病变均波及下腔静脉和右心房,其中1例肺动脉受累。1例患者发生肺内多发转移。结论：IVL的多排CT表现有一定特征性,9例患者均显示下腔静脉内充盈缺损延伸入右心房,CTV检查清晰地显示了IVL向子宫外生长的途径,有助于手术方案的制定。展开更多
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。
文摘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 l
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Our knowledge of renal cell carcinoma(RCC) is rapidly expanding. For those who diagnose and treat RCC, it is important to understand the new developments. In recent years, many new renal tumors have been described and defined, and our understanding of the biology and clinical correlates of these tumors is changing. Evolving concepts in Xp11 translocation carcinoma, mucinous tubular and spindle cell carcinoma, multilocular cystic clear cell RCC, and carcinoma associated with neuroblastoma are addressed within this review. Tubulocystic carcinoma, thyroid-like follicular carcinoma of kidney, acquired cystic disease-associated RCC, and clear cell papillary RCC are also described. Finally, candidate entities, including RCC with t(6;11) translocation, hybrid oncocytoma/chromophobe RCC, hereditary leiomyomatosis and RCC syndrome, and renal angiomyoadenomatous tumor are reviewed. Knowledge of these new entities is important for diagnosis, treatment and subsequent prognosis. This review provides a targeted summary of new developments in RCC.
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.
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 excisi
文摘BACKGROUND Leiomyomatosis peritonealis disseminata(LPD)is a rare condition characterized by multiple pelvic and abdominal nodules,which are composed of smoothmuscle cells.To date,no more than 200 cases have been reported.The diagnosis of LPD is difficult and there are no guidelines on the treatment of LPD.Currently,surgical excision is the mainstay.However,hormone blockade therapy can be an alternative choice.CASE SUMMARY A 33-year-old female patient with abdominal discomfort and palpable abdominal masses was admitted to our hospital.She had undergone four surgeries related to uterine leiomyoma in the past 8 years.Computed tomography revealed multiple nodules scattered within the abdominal wall and peritoneal cavity.Her symptoms and the result of the core-needle biopsy were consistent with LPD.The patient refused surgery and was then treated with tamoxifen,ulipristal acetate(a selective progesterone receptor modulator),and goserelin acetate(a gonadotropin-releasing hormone agonist).Both tamoxifen and ulipristal acetate were not effective in controlling the disease progression.However,the patient achieved an excellent response when goserelin acetate was attempted with relieved syndromes and obvious shrinkage of nodules.The largest nodule showed a 25%decrease in the sum of the longest diameters from pretreatment to posttreatment.Up to now,2 years have elapsed and the patient remains asymptomatic and there is no development of further nodules.CONCLUSION Goserelin acetate is effective for the management of LPD.The long-term use of goserelin acetate is thought to be safe and effective.Hormone blockade therapy can replace repeated surgical excision in recurrent patients.
文摘目的：由于子宫静脉内平滑肌瘤病（IVL）属于少见病例,其CT影像学表现有一定的特征性,回顾性分析IVL的多排CT特征及其临床表现,评价其对手术治疗的指导意义。方法：回顾性总结分析2005年至2013年间,通过外科手术及病理证实的9例IVL的患者的临床资料,多排CT特征以及病理结果。多排CT扫描参数：管电压100~120 k V,管电流400~450 m As,层厚0.5~0.75 mm,间隔0.25~0.60 mm。监测层面放在下腔静脉右心房入口区域,手动触发开始扫描。结果：9例患者中,4例患者为子宫切除术后,1例为多发子宫肌瘤并伴子宫腺肌病。多排CT均显示蛇形的下腔静脉内充盈缺损延伸入右心房,其中1例病变延伸入右心室、肺动脉。8例病例侵犯单侧宫旁血管,1例病例侵犯双侧子宫血管,6例肿瘤沿着子宫静脉、髂静脉生长,3例病例显示病变同时波及卵巢静脉和髂静脉,9例病变均波及下腔静脉和右心房,其中1例肺动脉受累。1例患者发生肺内多发转移。结论：IVL的多排CT表现有一定特征性,9例患者均显示下腔静脉内充盈缺损延伸入右心房,CTV检查清晰地显示了IVL向子宫外生长的途径,有助于手术方案的制定。
