Inferior vena cava and right atrial thrombosis in children with nephroblastoma: diagnostic and therapeutic problems

BACKGROUND: The neoplastic thrombus in Wilms' tumor rarely can extend in to the inferior vena cava or to the right atrium. The neoplastic thrombus usually is diagnosed concurrently with the tumor, although in some cases the diagnosis of the thrombus may precede the diagnosis of nephroblastoma. METHODS: Among 90 children with Wilms' tumor who were treated in the authors' unit, 4 had extensive tumor thrombosis of the inferior vena cava or the right atrium. One of these patients was found with a life-threatening thrombosis of the inferior vena cava and the right atrium, which was treated surgically; in this case, the diagnosis of nephroblastoma was made postoperatively. As for the 3 remaining patients the diagnosis of neoplastic thrombosis and Wilms' tumor was made simultaneously. RESULTS: In the first case, the patient underwent surgical excision of the thrombus with cardiopulmonary bypass and a short period of hypothermic cardiopulmonary arrest. In the other 3 cases the thrombus resolved with chemotherapy only. CONCLUSIONS: Surgical excision of extensive neoplastic thrombosis is suggested in the case of life-threatening thrombosis even with cardiopulmonary bypass. Chemotherapy is suggested in cases lacking clinical symptoms of thrombosis.     

Suprarenal vena caval occlusion. Principles of operative management.

Retrohepatic occlusion of the inferior vena cava caused by tumor complicates complete resection and not infrequently is associated with life-threatening symptoms that accelerate the lethality of the underlying malignant process. This report summarizes our experience with caval thrombectomy and reconstruction that allowed complete removal of all gross tumor in seven patients with malignant occlusion of the retrohepatic inferior vena cava. Included in this group are five patients with renal cell carcinoma and extension of tumor into the retrohepatic vena cava. Three of these patients had extension of tumor thrombus into the right atrium. A sixth patient had recurrent right adrenal cortical carcinoma with tumor invasion of the vena cava and occlusion to the right atrium. Associated hepatic vein occlusion and secondary Budd-Chiari syndrome also was successfully managed in this patient. The final patient with occlusion of the entire suprarenal vena cava required caval reconstruction after resection of a primary leiomyosarcoma of the retrohepatic portion of the vena cava. Careful planning of the operative procedure, adequate exposure, complete mobilization of the retrohepatic vena cava, and control of the hepatic venous effluent will allow patients with retrohepatic vena caval occlusions to be managed with safety and success.     

Thymic carcinoma with tumor thrombus into the superior vena cava

Tumor thrombus into the vena cava have been reported in cases with renal cell carcinoma, thyroid tumor and in those with thymoma. These tumors are frequently invasive and continuous from the main tumor that shows direct vessel wall invasion. Here, we report a case of thymic carcinoma with superior vena cava syndrome, which was caused by a tumor thrombus in the superior vena cava without vessel wall invasion. The main mediastinal tumor did not show innominate vein invasion, and the superior vena cava syndrome was a result of separate tumor thrombus that was free of vessel wall invasion. The tumor thrombus could be removed through a simple venotomy. To prevent stenosis in the superior vena cava and the left innominate vein, we used a pericardial patch to close the venotomy site.     

腹腔镜下微创手术治疗肾癌合并高位肝后下腔静脉癌栓

目的探讨腹腔镜下微创手术治疗肾癌合并高位肝后下腔静脉癌栓的临床经验和文献分析。 方法女性患者,61岁,临床诊断：右肾癌合并高位肝后下腔静脉癌栓。术前全面评估手术风险,组织多学科会诊为患者制定详尽的围手术期治疗与护理方案,拟行腹腔镜下右侧肾癌根治性切除+高位肝后下腔静脉癌栓取出+腹膜后淋巴结清扫术。术后医护密切配合严密观察患者病情变化,进行围手术期观察处理与护理。 结果手术顺利完成,手术时间390 min,无中转开放手术。术中完全游离右侧和左侧肾静脉、肝后下腔静脉直达第二肝门水平远端,近右肾静脉处下腔静脉内侧壁剪开静脉壁,癌栓下部小灶性侵犯静脉壁,切除部分腔静脉壁完整取出癌栓,恢复左侧肾静脉、腔静脉血流回流无障碍。术后病理提示符合透明细胞癌,癌组织侵犯肾窦脂肪,腹膜后淋巴结（-）。术后随访6个月未见肿瘤复发。 结论腹腔镜下微创手术治疗肾癌合并高位肝后下腔静脉癌栓安全可行,多学科协助模式为疑难复杂病例提供了一种新的选择,值得临床进一步推广。     

Assessment of tumor invasion of the vena caval wall in renal cell carcinoma cases by magnetic resonance imaging

PURPOSE: We evaluated the role of magnetic resonance imaging (MRI) in patients with renal cancer and inferior vena caval involvement with reference to its ability to characterize the extent and nature of inferior vena caval tumor extension and wall invasion. MATERIALS AND METHODS: The study included 12 consecutive patients with renal cancer and inferior vena caval involvement. All patients underwent imaging on a 1.5 Tesla MRI unit. Coronal, axial T1 and axial T2-weighted images were performed in all cases, while in 6 3-dimensional gadolinium enhanced magnetic resonance angiography and venography were also performed. Images were assessed for the extent and nature of tumor extension, that is tumor versus thrombus, and invasion of the inferior vena caval wall. Imaging results were compared with operative findings. RESULTS: On MRI the extent and nature of the inferior vena caval tumor was correctly defined in all cases. The sensitivity, specificity and accuracy of inferior vena caval wall invasion were 100%, 89% and 92%, respectively. CONCLUSIONS: In patients with renal cancer and inferior vena caval involvement MRI defines the tumor level in the inferior vena cava. It is also a sensitive technique for detecting vessel wall invasion and provides important preoperative information for surgical planning.     

Multimodal treatment of advanced adult Wilms tumor

We report 2 cases of advanced adult Wilms tumor that were treated with surgery, radiation and chemotherapy. The first patient had relapse of a Wilms tumor in the liver 2 years after nephrectomy. Combination chemotherapy, consisting of actinomycin D and vincristine, radiation therapy and final resection of the liver metastasis were successful and the patient has been free of disease for 4 years. The second patient had undergone transcatheter embolization of the renal artery elsewhere with the tentative diagnosis of an inoperable renal cell carcinoma metastatic to both lungs. A left renal tumor, weighing 4,500 gm., and a tumor thrombus in the vena cava extending to the right atrium were removed, and histologically diagnosed as a Wilms tumor. Subsequent chemotherapy and radiotherapy resulted in complete disappearance of the lung metastases. We conclude that multimodal treatment, namely a well timed combination of surgery, chemotherapy and radiotherapy, could potentially eradicate the disease even at an advanced stage.     

Transesophageal echocardiography in the surgical management of renal cell carcinoma with intracardiac extension.

Renal cell carcinomas may extend into the vena cava and the tumor thrombus occasionally involves the right atrium. The operative approach depends upon precise preoperative and intraoperative staging and thrombus localization. We report a case of renal cell carcinoma with complete inferior vena caval and hepatic vein occlusion with tumor extension into the right atrium. Preoperatively, transesophageal echocardiography provided superior images of the tumor and its extension, and intraoperatively allowed continuous monitoring of cardiac function and the removal of tumor from the atrium and inferior vena cava. Its use obviated the need for more costly and invasive preoperative and intraoperative procedures.     

Magnetic resonance imaging of renal carcinoma with extension into the vena cava: staging accuracy and recent advances.

In 16 patients with surgical confirmation of inferior vena cava thrombi from renal carcinoma, magnetic resonance imaging (MRI) and computed tomography (CT) were compared to assess the ability of non-invasive, cross-sectional imaging techniques to detect tumour thrombus, the level of its extension, and vessel wall invasion. MRI accurately detected tumour thrombus in the inferior vena cava in all 16 cases (CT in 14) and demonstrated the cephalad extent of tumour thrombi in 15 of 16 (CT in 11). The level of extension was more easily seen on MRI, as was the presence of extension into the hepatic veins--a finding not detected by CT. Tumour invasion of the wall of the inferior vena cava was correctly demonstrated in 7 cases on MRI and in 1 case on CT. In 8 of 16 patients, gradient recall acquisition in steady state (GRASS imaging) was performed in addition to conventional spin echo sequences and it correctly identified the composition of thrombus in all: tumour in 6 patients, blood clot in 1 and both tumour and blood clot in the remaining patient. MRI and CT are excellent for detection of tumour but MRI is superior in the evaluation of vascular extension. In addition, the use of GRASS imaging allows differentiation of tumour from blood thrombus.     

肝癌合并下腔静脉癌栓的外科治疗

目的总结7例肝癌合并下腔静脉（inferior vena cava,IVC）癌栓患者的手术方法及治疗经验。方法自2003年7月至2005年5月,我们为7例肝癌合并IVC癌栓的患者实施了肝癌切除及右心房和（或）IVC切开取栓手术。所有患者均采用全肝血流阻断来控制IVC血流。根据癌栓上极位置的不同,分别采用5种不同术式：（1）静脉转流,心脏停搏,右心房及下腔静脉切开取栓1例;（2）静脉转流,心脏不停搏,心包内高位阻断下腔静脉,右心房和（或）下腔静脉切开取栓2例;（3）经腹部切口切开膈肌,心包内高位阻断下腔静脉,下腔静脉切开取栓1例;（4）经腹部切口,经膈肌腔静脉裂孔小切口,心包外高位阻断肝上下腔静脉,下腔静脉切开取栓1例;（5）经腹部切口,肝上阻断下腔静脉,下腔静脉切开取栓2例。结果所有手术均获成功,术后并发症包括胸腔积液2例,右膈下积液1例,切口感染1例。7例患者的生存时间为2周～26个月,平均9．8个月。已死亡的6例患者术后生存时间分别为13、9、11、2、17个月和2周,尚生存的1例患者已无瘤生存26个月。结论对合适病例实施肝癌切除和IVC切开取栓手术是安全可行的。手术治疗可以避免右心流人道阻塞和肺动脉栓塞造成的猝死,并有可能获得相对提高的生存时间和生活质量。     

Diagnostic and therapeutic approach to the extension of kidney cancer to the vena cava

In the light of their experience with 16 cases seen over 5 years, the authors analyze the diagnostic and therapeutic tools for assessment of the vena cava thrombi that complicate 5 to 10% of renal carcinomas. Cavography still plays a central part in the detection of vena cava lesions. Localization of the upper extremity of the thrombus is needed to decide upon the operative technique and can be achieved by free flow inferior cavography for free floating thrombi; for complete thrombi, the two most informative procedures appear to be echocardiography (to evaluate the right atrium and intrathoracic inferior vena cava) and inferior cavography by the superior route; it seems that computed tomography provides no additional information in the assessment of extensive spread to the inferior vena cava. 14 patients were treated surgically: the surgical approach is dictated by the location of the thrombus and should allow control of the vena cava proximal to the thrombi. Two patients with a thrombus that extended into the right atrium had surgery using extracorporeal circulation; because this method is especially safe, extension of its indications to retrohepatic thrombi may be justified. The absence of operative mortality and comparison of results to those previously reported in the literature confirm the value of surgical treatment of vena cava lesions, especially if there is no lymph node involvement.     

Tumor thrombectomy for endometrial stromal sarcoma extending into the inferior vena cava and the right atrium from the uterus

We report an extremely rare case of endometrial stromal sarcoma (ESS) extending into the inferior vena cava and the right atrium. A 65-year-old woman was admitted to our hospital due to lower-extremity edema. The chest-abdominal computed tomography (CT) showed tumor thrombus invading the inferior vena cava and right atrium with multiple lung metastasis. To prevent sudden death from pulmonary embolism, she underwent surgical removal the tumor thrombus with the use of cardiopulmonary bypass and deep hypothermic circulatory arrest. The pathological diagnosis of the tumor thrombus was low-grade ESS originating from the uterus. After thrombectomy, she underwent chemotherapy with carboplatin and paclitaxel. Surgical resection and chemotherapy to low-grade ESS achieved favourable prognosis.     

Extension of renal cell carcinoma into the vena cava: clinical review and surgical approach

Between 1972 and 1983, 25 patients were treated for renal carcinoma with tumor extension into the vena cava but without other clinical evidence of disseminated disease. Of these patients 12 had vena caval tumor thrombus extension up to the level of the hepatic veins (group 1), 10 had extension into the intrahepatic vena cava (group 2) and 3 had tumor extending into the right atrium (group 3). A perioperative management plan and an anatomical surgical approach have been developed to allow safe en bloc removal of these extensive tumor thrombi without removal of the vena cava. Successful management is dependent upon preoperative evaluation to determine precisely the extent of the disease, prophylaxis against pulmonary embolism and a well planned surgical method. For patients without evidence of metastatic or perinephric disease, the 5-year actuarial survival rate of 33 per cent is comparable to that of other patients without thrombus. Complete resection was possible in 20 patients (80 per cent), with a 5-year actuarial survival rate of 36 per cent. While patients with metastatic tumor cannot be cured, short-term palliation can be achieved for patients who have an imminent risk of vena caval occlusion or pulmonary embolism by an en bloc removal of tumor and thrombus, even for those with intra-atrial extension. Over-all, operative intervention was successful, with 22 of 25 patients leaving the hospital alive.     

TEE bei intracavaler Ausdehnung von Nierenzellkarzinomen Intraoperatives Management mit transösophagealer Echokardiographie

OBJECTIVE: To evaluate the role of intraoperative real-time transesophageal echocardiography (TEE) for the anesthetic and surgical management of patients with renal cell carcinoma and vena cava extension. METHODS: Retrospective analysis of the intraoperative application of TEE in a series of 4 patients. RESULTS: Real-time TEE with a multiplane probe allowed visualization of inferior vena cava tumor extensions, accurate assessment of the distal extent of vena cava invasion into hepatic veins and right atrium, monitoring of embolism and evaluation of cardiac preload and function in all patients. CONCLUSION: Intraoperative TEE is a useful adjunct to the anesthetic and surgical management of patients with renal cell carcinoma and vena cava extension.     

The removal of renal carcinoma thrombus extending to the right atrium by means of extracorporeal circulation: report of a case]

A case of renal cell carcinoma with a tumor thrombus extending to the right atrium was reported. A 70-year-old woman was admitted with a diagnosis of right renal tumor which had been detected on a routine abdominal ultrasonography. MRI revealed a tumor thrombus extending into the right atrium through the inferior vena cava. A transesophageal echocardiogram confirmed that the tumor extended into the right atrium, and was not adherent to the inferior vena cava and the atrium. Right nephrectomy and removal of the tumor thrombus were performed using extracorporeal circulation. Temporary occlusion of portal venous and hepatic arterial inflow was effective in reducing blood loss. She has been doing well, and there has been no evidence of recurrence during 18 month postoperatively.     

Intravascular Extension of Wilms Tumor

OBJECTIVE: To define the incidence and manifestations of and optimal therapy for children with intravascular extension of Wilms tumor. METHODS: Children on a collaborative study of Wilms tumor who had intravascular extension into the inferior vena cava (IVC) or atrium were identified. Surgical checklists and surgical and pathology reports were reviewed. RESULTS: One hundred sixty-five of 2,731 patients had intravascular extension of Wilms tumor. The level of extension was IVC in 134 and atrium in 31. Sixty-nine had received preoperative therapy (55 with IVC extension and 14 with atrial extension) for a median of 8 weeks. Complications during preoperative chemotherapy were seen in five patients (tumor embolism and tumor progression in one each, and three with adult respiratory distress syndrome, one of which was fatal). The intravascular extension of the tumor regressed in 39 of 49 children with comparable pre- and posttherapy radiographic studies, including 7 of 12 in whom the tumor regressed from an atrial location, thus obviating the need for cardiopulmonary bypass. Surgical complications occurred in 36.7% of the children in the atrial group and 17.2% in the IVC group. The frequency of surgical complications was 26% in the primary resection group versus 13.2% in children with preoperative therapy. When all the complications of therapy were considered, including those that occurred during the interval of preoperative chemotherapy (one of the five also had a surgical complication), the incidence of complications among those receiving preoperative therapy was not statistically different from the incidence among those who underwent primary resection. The difference in 3-year relapse-free survival (76.9% for 165 patients with intravascular extension, 80.3% for 1,622 patients with no extension) was not statistically significant whether or not it was adjusted for stage and histology. CONCLUSIONS: Preoperative treatment of these children may facilitate resection by decreasing the extent of the tumor thrombus, but the overall frequency of complications is similar in both groups.     

NMR tomography of vena cava thrombi: clinical significance in the differential diagnosis and therapy

31 patients with advanced renal cell carcinoma underwent abdominal MRI which were examined by an independent experienced radiologist and compared with the intraoperative findings. 12 of 13 vena caval tumor thrombi were diagnosed correctly, 1 small tumor thrombus in a case with large retroperitoneal lymph nodes compressing the vena cava could not be detected. The extension of the tumor in the vena cava could exactly be described in topographic relation to liver, diaphragm and right atrium which is important for planning the operative procedure.     

Magnetic resonance imaging and preoperative evaluation of cancer of the kidney. The results apropos of 60 cases

Sixty renal carcinomas confirmed at surgery or autopsy were studied. Capsular effraction, present in 17 cases, was well assessed in 8 cases, under staged in 8 cases and over staged in 5 cases (sensitivity 47%, specificity 88%). Renal vein involvement was present in 11 cases. In 8 of these 11 cases, a thrombus was present in the inferior vena cava. MRI detected a thrombus in the renal veins in 10/11 cases and in 7/8 cases of caval invasion. The false negative case was due to a huge right upper pole tumor laminating the inferior vena cava. The false positive case was due to an enlarged lymph node compressing the inferior vena cava. Cranial extension of the thrombus was well assessed in 6 of the 7 cases. One thrombus in the right atrium was missed. Lymph node involvement was present in 10 cases and correctly diagnosed by MRI in 7 cases. Three false negative cases were noted, because of microscopic invasion in non enlarged lymph nodes. Adjacent organ invasion, present in 2 cases, was detected in 1 case of liver invasion. Initial results of MRI seem very promising and at present, the best indications of MRI in pre-operative evaluation of a renal carcinoma are assessment of caval extension and spread to adjacent organs in patients with large tumors.     

肾癌并静脉癌栓的影像学诊断与手术方法选择

目的：探讨肾癌并静脉癌栓的影像学诊断与治疗及方法的选择。方法：回顾性分析我科收治的肾癌伴静脉癌栓患者21例的临床资料。结果：MRI精确地诊断出癌栓的范围；20例肾癌根治性切除加癌栓取出术的患者取得了满意的效果。结论：MRI可替代创伤性大、不良反应多的下腔静脉造影，用于确诊肾癌并静脉癌栓；应依据癌栓的类型选择手术方法。     

A strategy for resection of Wilms' tumor with vena cava or atrial extension.

Resection of a Wilms' tumor that extends into the vena cava or right atrium results in excellent survival when combined with adjuvant therapy. Preoperative identification of the presence of intravascular tumor thrombus and the level of vascular involvement is essential. It facilitates safe surgical resection, with cardiopulmonary bypass immediately available for retrohepatic and atrial tumors. Six patients with intracaval or intracardiac tumor thrombus were treated over a 5-year period with no perioperative deaths. Preoperative chemotherapy was useful in two patients with extensive tumors and pulmonary metastases. Our results using an integrated management plan suggest that an aggressive surgical approach is justified for this extensive variant of Wilms' tumor.     

Renal cell carcinoma extending into the right atrium; report of a case

A 62-year-old male was diagnosed through abdominal ultrasonography, with right renal cell carcinoma extending into the inferior vena cava. Surgery was performed because echocardiography revealed the tumor to have reached the right atrium. The portion of the tumor situated in the right atrium was resected under the extracorporeal circulation. Distal part of inferior vena cava was resected with the tumor included. The tumor remaining in the confluence of hepatic veins was removed from the incised end of the inferior vena cava and was detached from the venous wall. Postoperative abdominal echography revealed a small additional tumor mass in hepatic veins. Although this mass was considered to be a remnant of the intravenous tumor, an additional surgical procedure was judged to be impossible. In retrospect, an additional long-axis incision on the inferior vena cava might have enabled us to catch the remnant of the tumor thrombus in the hepatic vein.