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A: Surgical incision; black arrow: median sternotomy, black dotted arrow: bilateral subcostal incision with superior midline T-extension. B: Tumor-thrombus extension from right renal vein to right cardiac chamber. Black arrow: mobilized liver.
Source publication
Renal cell carcinoma is a tumor with the distinct feature that it can invade through the renal vein into the inferior vena cava, and can grow intravascularly, sometimes extending into right cardiac chambers. Surgical resection provides the only reasonable chance for a cure, and cardiopulmonary bypass with hypothermic circulatory arrest is used to r...
Contexts in source publication
Context 1
... was performed in collabora- tion with a urological team. During surgery, the tumor- thrombus was monitored with transesophageal echo- cardiography (TEE). Both patients underwent the same operation, involving the following basic steps. The right kidney was mobilized through a bilateral subcostal inci- sion with superior midline T-extension ( Fig. 2A), leaving only the vein tethering the kidney (the right renal artery and the right ureter were ligated and sectioned). The liver was mobilized too. The incision was extended from the xiphoid to the sternal notch ( Fig. 2A). Cardiopulmonary bypass (CPB) was established as usual; arterial cannula (20 Fr) was placed in the ascending aorta, ...
Context 2
... basic steps. The right kidney was mobilized through a bilateral subcostal inci- sion with superior midline T-extension ( Fig. 2A), leaving only the vein tethering the kidney (the right renal artery and the right ureter were ligated and sectioned). The liver was mobilized too. The incision was extended from the xiphoid to the sternal notch ( Fig. 2A). Cardiopulmonary bypass (CPB) was established as usual; arterial cannula (20 Fr) was placed in the ascending aorta, venous can- nulas were placed in the superior vena cava (24 Fr) and right femoral vein (21 Fr). The patient was cooled to 22°C (nasopharyngeal temperature); immediately before hypothermic circulatory arrest (HCA), the ...
Context 3
... (HCA), the ascending aorta was cross clamped, and cold blood cardioplegic solution was administered for myocardial protection antegradely. Cell-saving techniques were not used for a blood aspiration. The right atrium (RA) was opened near the orifice of the IVC; the bloodless field allowed for complete intravascular tumor-thrombus extirpation (Fig. 2B). The tumor-thrombus was transected at the hepatic vein level; an incision was made around the origin of the right renal vein and extended cephalad along the IVC. Gentle traction then permitted en bloc removal of the kidney and remaining thrombus. After the IVC and RA were closed, the patient was weaned from CPB and cannulas were ...
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Background
In the advanced stages of hepatocellular carcinoma (HCC), a tumor thrombus (TT) can form in the portal or hepatic vein. The management of patients with advanced HCC and a TT extending into the right atrium (RA) and inferior vena cava (IVC) is extremely difficult and risky. Case presentationWe report the case of a patient with HCC and a l...
Background: Renal cell carcinoma (RCC) has a tendency to invade the renal venous system with tumor thrombus. In this study, we assessed the surgical strategy for RCC with tumor thrombus, especially those expanding into the inferior vena cava (IVC). Patients and Methods: We retrospectively reviewed our clinical records for all patients who underwent...
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Citations
... Symptoms associated with RCC TT presentation include varicocele, lower extremity swelling, cardiac dysfunction, pulmonary embolism, or Budd-Chiari syndrome. However, in some instances, TT may remain asymptomatic and only be incidentally discovered through imaging [14][15][16][17][18]. Despite extensive documentation of RCC TT in the US, limited information exists regarding its presentation, management, and outcomes in Puerto Rico (PR). ...
... According to the literature, the presentation of TT in RCC patients is variable; some remain asymptomatic, while others exhibit symptoms like varicocele, lower extremity swelling, cardiac dysfunction, pulmonary embolism, or Budd-Chiari Syndrome [14][15][16][17][18]. In our patient cohort, as shown in Table 3, none displayed symptoms indicative of a TT, except for one case of pulmonary embolism (PE). ...
Introduction: Renal cell carcinoma (RCC) is one of the most common types of kidney cancer. While RCC tends to present as a localized tumor, a notable proportion may present with distant metastasis. In some instances, RCC may also present with intravascular tumor extension, often called tumor thrombus (TT). Its presence confers a worse prognosis and has important implications for the tumor's staging and treatment. Despite extensive documentation of RCC TT in the US, limited data exists regarding its presentation, management, and outcomes in Puerto Rico (PR). This study aims to broaden the available information on RCC TT, emphasizing surgical management and outcomes. We also provide descriptive data on patient demographics and clinical presentation to improve decision-making among clinicians caring for Puerto Rican men and women.
Methods: In this single-center, retrospective study, we evaluated patients who underwent partial or total nephrectomy at Saint Luke's Episcopal Medical Center between 2018 and 2022. Data was abstracted from electronic health records (EHR). Patients without documented evidence of TT during the peri-operative period were excluded from the study. A total of 220 patient records were evaluated, of which 12 met the inclusion criteria for the study. Cases were categorized using the latest RCC TT guidelines. Central tendency measurements were used to describe the sample distribution. The mean was considered to make assumptions regarding the prevalent observations, and the median was considered to rule out possible outliers. Categorical data were evaluated using proportion analyses, including TT extension level and BMI variables. Fisher’s exact test evaluated the association between the World Health Organization/International Society of Urological Pathology (WHO/ISUP) grade and TT extension level.
Results: Most patients lacked TT-related symptoms. The most severe presenting symptom was a pulmonary embolism (8.3%). Hypertension (83.3%), BMI greater than 25 at the time of diagnosis (75%), and type 2 diabetes mellitus (66.7%) were the most common comorbid conditions within our cohort. Nearly 75% of patients underwent laparoscopic radical nephrectomy with TT resection. One left-sided level III case was managed by laparoscopic-assisted open radical nephrectomy with a right subcostal incision. There were zero intraoperative complications and two postoperative complications. The histopathological reports of all cases were consistent with clear cell carcinoma, and half of the cases (n=6) were WHO/ISUP G4. All patients are alive and free of disease.
Conclusion: RCC is a common renal neoplasm in PR that can present with intravascular tumor extension. Our findings do not establish a definitive association between BMI, tumor size, WHO/ISUP grading, and TT extension level. Our study shows that laparoscopic removal of RCC TT is a safe and effective approach. However, the generalizability of our findings is limited by the study's design and sample size. Future research should focus on identifying predictive markers, establishing effective screening protocols, and determining if our hybrid approach has comparable outcomes to the standard open approach.
... Renal cell carcinoma (RCC) is responsible for over 180,000 deaths worldwide, and accounts for 2.4% of all cancer diagnoses worldwide [1]. RCC with tumor thrombus extension occurs in 5-15% of RCC cases and requires prompt intervention [2]. Mayo Level IV thrombus, defined as a thrombus extending above the diaphragm or into the right atrium, occurs in 1% of cases and is particularly life-threatening, traditionally requiring urgent surgical management [3]. ...
Background: Renal cell carcinoma (RCC) tends to undergo intravascular tumor growth along the renal vein, forming tumor thrombi that may extend into the inferior vena cava (IVC) or even the right atrium (Level IV). Managing such cases requires a multidisciplinary approach, especially in patients with acute coronavirus disease 2019 (COVID-19) infection, who face increased risks from surgical interventions. We present a case of RCC with Level IV thrombus and concurrent COVID-19 managed with systemic therapy. We also summarize current literature on treating RCC with IVC thrombus and COVID-19's impact on prognosis.
Case Presentation: The patient was a 70-year-old female with incidental detection of a 9-cm right heterogeneous renal mass with a supradiaphragmatic tumor thrombus during COVID-19 infection. Due to ongoing pulmonary symptoms, systemic therapy with a combination of ipilimumab and nivolumab was initiated. After an excellent initial response, the patient continued systemic therapy, maintaining a necrotic response in the renal mass and tumor thrombus. The patient continues to tolerate systemic therapy well.
Conclusion: We report a rare case of RCC with Level IV tumor thrombus and synchronous acute COVID-19 infection. Our report depicts successful management utilizing systemic therapy with a combination of ipilimumab and nivolumab. The management of such cases necessitates a comprehensive, multidisciplinary approach, considering the risks associated with surgery in the context of recent COVID-19 infection. The case presentation and ensuing literature discussion of the dynamic landscape of RCC management highlights the need for more research to improve treatment plans and guide clinicians in handling such complex situations.
... Type 0 is limited to the renal vein, type I involves infiltration of the IVC less than 2 cm above the renal vein, and type II extends to the IVC below the hepatic veins. Type III is defined as the progression of TT up to the hepatic veins but below the diaphragm, and type IV extends above the diaphragm and includes the right cardiac atrium [4,14]. The absence of TT has been shown to have a statistically significant impact on improved survival outcomes [10]. ...
Simple Summary
Tumor thrombus occurs when tumor cells extend into a blood vessel. An estimated 10% of kidney cancer cases are complicated by tumor thrombus, often invading the renal vein with extension to the inferior vena cava. Up to 1% have tumor cells extending to the heart. The standard of care for these patients is surgical removal of the kidney tumor and the tumor thrombus. Research focuses on surgical techniques, imaging methods, and molecular markers for prognosis. The full benefit of anticoagulation remains controversial in these cases, considering unknown benefits and bleeding risk during tyrosine kinase inhibitor therapy. In this literature review, we summarize known data regarding the use of anticoagulation in the setting of kidney cancer and tumor thrombus.
Abstract
Tumor thrombus (TT) is a complication of renal cell carcinoma (RCC) for which favorable medical management remains undefined. While radical nephrectomy has been shown to increase overall survival in RCC patients, surgical interventions such as cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA) utilized to perform TT resection carry high mortality rates. While it has been documented that RCC with TT is associated with venous thromboembolism (VTE) development, anticoagulation use in these patients remains controversial in clinical practice. Whether anticoagulation is associated with improved survival outcomes remains unclear. Furthermore, if anticoagulation is initiated, there is limited evidence for whether direct oral anticoagulants (DOACs), heparin, or warfarin serve as the most advantageous choice. While the combination of immunotherapy and tyrosine kinase inhibitors (TKIs) has been shown to improve the outcomes of RCC, the clinical benefits of this combination are not well studied prospectively in cases with TT. In this literature review, we explore the challenges of treating RCC-associated TT with special attention to anticoagulation. We provide a comprehensive overview of current surgical and medical approaches and summarize recent studies investigating anticoagulation in RCC patients undergoing surgery, targeted therapy, and/or immunotherapy. Our goal is to provide clinicians with updated clinical insight into anticoagulation for RCC-associated TT patients.
... RCC can invade through the renal vein into the IVC, and can then extend into the lumen, with tumour-thrombus complex formation occurring in about 5% to 15% of all cases. The tumour can sometimes extend right up to the right cardiac chambers as well from there in about 1% of the cases [5]. There are four stages of this tumour thrombus extension to the heart; type I involves the intravascular tumour reaching the renal vein but not the IVC, type II involves the IVC being occupied up to the level of the hepatic veins, while in types III and IV the IVC above the diaphragm and, subsequently, the right cardiac chamber are involved [6]. ...
Renal cell carcinoma (RCC) is an aggressive tumour, with 25% of the cases presenting with distant metastases at the time of diagnosis. Approximately 33% of the patients with RCC eventually develop metastatic spread. RCC can metastasize to various sites including the lung, liver, bone, brain, adrenal gland, and more. Cardiac metastasis is rare in RCC, but even rarer in the absence of inferior vena cava (IVC) involvement. This case report presents a 60-year-old male patient who was referred by his general practitioner due to breathing difficulties. An initial echocardiogram revealed a right ventricular outflow tract obstruction caused by a mass. A subsequent cardiac MRI showed a right ventricular mass with features suggestive of a metastatic spread. A CT scan of the thorax, abdomen and pelvis was done to ascertain the primary tumour which revealed RCC, without involving the IVC. Due to the presence of metastases, advanced disease, and heavy tumour burden, the multidisciplinary team concluded that there were almost negligible treatment options available at that stage and recommended the best supportive care and community hospice support. The patient was discharged once his symptoms improved, as per his request, and he passed away peacefully at home within a month. This case highlights the very rare occurrence of cardiac metastasis of RCC without IVC involvement. It also illustrates the approach and investigations involved in the evaluation of complex cardiac masses.
... In addition, RN is applicable in the treatment of IVC. It is performed at stage I and II of IVC invasion, while stage III and IV require additional methods to achieve a bloodless surgical field, and these include cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA) [29]. The use of these methods, while reducing the risk of perioperative death, carries a significant risk of perioperative complications [30]. ...
Simple Summary
Methods of treatment should be carefully considered. Systemic therapy is recommended for patients susceptible to this method, as it will allow them to avoid surgery and postoperative complications. Surgery remains the standard of care for operable RCC resistant to systemic therapy.
Abstract
The treatment of metastatic renal cell carcinoma has undergone considerable advances in the last two decades. Cytoreductive nephrectomy and metastasectomy retains a role in patients with a limited metastatic burden. The choice of optimal treatment regimen remains a matter of debate. The article summarises the current role of surgery in metastatic kidney cancer.
... Migration of the tumor is into the right atrium (RA) is an even rarer event and occurs in 1% of cases [2][3][4][5]. Tumor thrombus may present asymptomatically or cause a variety of symptoms, such as varicocele, lower extremity swelling, cardiac dysfunction, pulmonary embolism, or Budd-Chiari syndrome (BCS) [6,7]. The treatment of metastatic RCC is rapidly evolving and the role of immunotherapy is ongoing [8], however surgery remains the most effective form of treatment for RCC with TT [9][10][11][12]. ...
Renal cell carcinoma (RCC) with inferior vena cava (IVC) and right atrium (RA) tumor thrombus (TT) is a rare occurrence and its resection is surgical challenge. Management becomes even more difficult when the TT causes hepatic vein obstruction and leads to Budd-Chiari syndrome. We report a case of 68-year-old male with right RCC with IVC and RA TT with associated Budd-Chiari syndrome. Surgical management was performed without cardiopulmonary bypass (CPB) and re-sternotomy due to the patient's previous history of coronary artery bypass grafting (CABG) for 3 vessel coronary artery disease. Through a transabdominal approach, the diaphragm was dissected off the IVC and the RA was gently pulled into the abdomen and clamped under transesophageal echocardiogram (TEE) control. As use of CPB in these surgeries is associated with increased morbidity and mortality, this organ transplant-based approach is encouraged for patients requiring resection of RCC with supradiaphragmatic TT.
... There were more than 431,280 new kidney cancer cases diagnosed in 2020 worldwide [1], of which the most common histological subtype is clear cell renal cell carcinoma (ccRCC), accounting for approximately 70-80% of all renal cell carcinoma (RCC) cases [2][3][4][5]. One unique clinical aspect of RCC is that it can invade through the renal vein into the inferior vena cava (IVC), and even grow up to the right cardiac chambers [6]. Venous tumor thrombus (TT) in the renal vein or inferior vena cava was reported in approximately 15% of RCC patients [7]. ...
Background
Vascular invasion with tumor thrombus frequently occurs in advanced renal cell carcinoma (RCC). Thrombectomy is one of the most challenging surgeries with high rate of perioperative morbidity and mortality. However, the mechanisms driving tumor thrombus formation are poorly understood which is required for designing effective therapy for eliminating tumor thrombus.
Results
We perform single-cell RNA sequencing analysis of 19 surgical tissue specimens from 8 clear cell renal cell carcinoma (ccRCC) patients with tumor thrombus. We observe tumor thrombus has increased tissue resident CD8 ⁺ T cells with a progenitor exhausted phenotype compared with the matched primary tumors. Remarkably, macrophages, malignant cells, endothelial cells and myofibroblasts from TTs exhibit enhanced remodeling of the extracellular matrix. The macrophages and malignant cells from primary tumors represent proinflammatory states, but also increase the expression of immunosuppressive markers compared to tumor thrombus. Finally, differential gene expression and interaction analyses reveal that tumor-stroma interplay reshapes the extracellular matrix in tumor thrombus associated with poor survival.
Conclusions
Our comprehensive picture of the ecosystem of ccRCC with tumor thrombus provides deeper insights into the mechanisms of tumor thrombus formation, which may aid in the design of effective neoadjuvant therapy to promote downstaging of tumor thrombus and decrease the perioperative morbidity and mortality of thrombectomy.
... Por último el hipernefroma renal puede invadir las cavidades derechas cardiacas por invasión de la vena cava inferior a través de la vena renal en un 1% de la totalidad de estos tumores (6) . En nuestro caso no se objetivó invasión de la ...
Presentamos el caso de una mujer de mediana edad que ingresa como primer episodio de insuficiencia cardiaca en el servicio de cardiología de nuestro centro, siendo diagnosticada de miocardiopatía dilatada no isquémica con hallazgo de varios trombos biventriculares. Durante el seguimiento se demostró que uno de los factores protrombóticos asociados a este caso era un hipernefroma silente. Mediante este caso revisamos brevemente esta entidad con especial hincapié en las técnicas de imagen en el diagnóstico de estos
... At the time of initial diagnosis of RCC, 65% of patients have localized disease, 16% of patients have regional lymph node involvement, and 16% of patients have metastatic disease [2]. RCC can invade locally through the renal vein and into the inferior vena cava (IVC) with tumor-thrombus formation reported in 5%-15% of patients [3,4]. RCC involvement of the IVC can lead to ascites, pulmonary emboli, lower extremity edema, and many other complications. ...
... From the IVC, RCC can grow intravascularly and extend into the right atrium. RCC extension to the right side of the heart is a rare complication that occurs in approximately 1% of RCC patients [3,4]. RCC is a masquerader of all malignancies and can present with a wide range of clinical signs and symptoms. ...
... Tumor-thrombus formation is a rare complication of RCC that occurs in approximately 5%-15% of patients [3,4]. The tumor-thrombus extended to the right atrium, resulting in a large 6.9 cm x 3.8 cm solid mass that nearly filled the right atrium and obstructed the blood flow leading to severe functional tricuspid stenosis. ...
Renal cell carcinoma (RCC) can invade locally through the renal vein and into the inferior vena cava (IVC) with tumor-thrombus formation reported in 5%-15% of patients. From the IVC, RCC can grow intravascularly and extend into the right atrium. We present a rare case with two uncommon findings: tumor-thrombus extension leading to a right atrial mass and initial presentation of RCC as heart failure. A 69-year-old woman presented with signs and symptoms of heart failure. Electrocardiogram was normal and the initial troponin level was mildly elevated to 0.09 ng/mL. Echocardiography revealed a dilated right atrium with a 6.9 cm x 3.8 cm echogenic mass consistent with a tumor impinging on the tricuspid valve leading to a functional stenosis. Computed tomography (CT) of the abdomen revealed a large right-sided renal mass with enlargement of the renal vein suggestive of tumor thrombus. Although the initial presentation of RCC with cardiac symptoms is surprising, this case highlights the importance of maintaining a comprehensive differential diagnosis. It also signifies the need for further imaging as not all atrial masses are cardiac tumors. Many other primary tumors - kidney, liver, lung, and thyroid - can directly invade or metastasize into the atrium by way of the vena cava.
... Renal cell carcinoma should always be considered when it comes to IVC tumoral extension. Between 5% and 15% of renal cell neoplasms develop a tumoral thrombus that penetrates the renal vein and can occupy the IVC up to the right atrium, in 1% of the cases [24]. Usually, the histopathological result of renal cell carcinoma is the most frequent scenario, especially when associated with a voluminous renal mass. ...
Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare malignant tumor, accounting for 2% of all LMSs. Less than 400 cases have been reported in literature. Computed tomography (CT) is the most accurate imaging method in assessing the location of the tumor within the IVC and magnetic resonance imaging (MRI) accurately identifies its extent and the potential for surgical resection. We present the case of a patient with inferior vena cava leiomyosarcoma (IVCL), for whom the pathological diagnosis was different from the initially expected one, the tumor appearance on pre-operative imaging mimicking renal cell carcinoma. The intraoperative difficulty of approaching renal hilum and IVC was a factor suggesting the vascular origin of the tumor, which was confirmed at pathological analysis. The extensive defect in the IVC after tumor excision led to the decision of complete transverse suturing of IVC, as significant collateral venous circulation was already present. Because IVCL is a rare disease, there is scarce data regarding the prognosis and treatment options. Long-term survival depends on the extent of the surgery. The need of vascular reconstruction is not always mandatory. Despite high recurrence rates, no consensus regarding adjuvant treatment exists yet. A multidisciplinary approach including surgical oncologists and vascular surgeons is mandatory to achieve the best patient outcomes. Perioperative planning, coordination and adherence to oncological techniques are critical.
