Inferior Vena Cava Involvement Research Articles

Clinical outcomes after inferior vena cava resection for malignant disease. A single center experience of 51 vena cava resections

Background and objectiveFor tumors involving inferior vena cava (IVC), surgery with complete resection remains the first line treatment. Management of IVC after resection, either ligation without reconstruction or primary reconstruction, is debated. Our study aimed to evaluate type of venous reconstruction, anticoagulation management and morbidity. MethodsA French single center database of patients who underwent partial or total circumferencial resection of the IVC for malignant disease was analyzed. Inclusion criteria were any oncologic procedure for a retroperitoneal neoplasm requiring concomitant resection of the IVC with or without venous reconstruction with prosthesis. Exclusion criteria were surgery before year 2000. Data were descriptive and reverse Kaplan Meier was used for follow-up calculation. The endpoints were the rate of prosthetic reconstruction, the use of anticoagulation and the post-operative outcomes. ResultsFifty – one patients were included with a median duration of follow-up of 54.8 months. The majority of patients were men (56.9%). Median age of the population was 44.1 years. Most of the patients underwent surgery for primary testicular cancer and for sarcoma. Complete IVC resections were performed in 46 (90,2%) patients, 32 having a concomitant prosthetic replacement. Eight patients underwent aortic resection in the same operative time. Postoperative morbidity was 33.3%. Post-operative anticoagulation was done in 24 patients. At 1 month, four patients developed thrombosis in the prosthesis. ConclusionsIVC resections are feasible and safe. Venous reconstruction and postoperative management were planned according to the preoperative imaging and intraoperative findings. We propose a decision-tree for peri-operative management and anticoagulation.

Inoperable Renal Malignant Glomus Tumor, the answers for all the "W's"?

Glomus tumor, arising from glomus bodies (specialized neurovascular structures involved in thermoregulation), commonly occurs in extremities and rarely in viscera. The spectrum of glomus tumors range from benign tumors to tumors with uncertain malignant potential to tumors of the malignant subtype. A vast majority of visceral glomus tumors are benign. Most common visceral tumors arise in the gastrointestinal tract. Glomus tumors of the kidney are a rare entity of which malignant glomus tumors are exceedingly rare. The index patients in the existing case reports were middle-aged males. We report our experience with malignant glomus tumor of the left kidney in a 60-year-old female, with computed tomography (CT) showing involvement of renal vein and inferior vena cava (IVC). Percutaneous biopsy was performed as imaging did not conform to the appearance of a conventional renal tumor and was reported as malignant glomus tumor after immunohistochemistry. After informed decision, the patient and family elected to proceed with surgery. However, intraoperatively, the left renal mass was found to infiltrate the pancreas, duodenum, aorta, and root of the colonic mesentery due to which surgery was aborted. Biopsy obtained intraoperatively again confirmed diagnosis of left renal malignant glomus tumor. She had an uneventful postoperative recovery. Options of treatment were reviewed by a multidisciplinary board. In light of no proven benefit for systemic therapy, she was referred for supportive care. She was under follow-up and she expired after 7 months due to progressive disease. Our literature review focuses on the clinicopathologic features and the current standard of management of malignant renal glomus tumors.

Tumor characteristics associated with detectable circulating tumor DNA preoperatively in patients with renal masses suspicious for RCC.

461 Background: Understanding the specific tumor characteristics associated with detectable ctDNA in the blood of patients with renal cell carcinoma (RCC) is critical to informing future studies seeking to establish the clinical utility of such testing. We characterized pathological and clinical characteristics associated with ctDNA detected preoperatively in patients with renal masses suspicious of RCC. Methods: Using our single institution prospectively maintained database, we identified consecutive patients who underwent partial or radical nephrectomy for non-metastatic suspected RCC (stages cT1b-cT3) during 2022-2023. Included were patients who had undergone tumor-informed ctDNA testing using the commercial Signatera assay (Natera). Baseline characteristics, pathology results, imaging study results, and oncological treatment and follow-up data were collected from the electronic medical records. ctDNA results were collected through the Natera portal. Study findings were reported using descriptive statistics. A p-value of <0.05 was considered statistically significant. R programming language version 4.3 was used for all statistical analyses. Results: A total of 54 patients with a median age of 63 years (IQR 51-71) were included in the study. Twenty-one (39%) were women. The median follow-up time was 4 months (range: 1-21 months). Among the 54 patients, 27 (50%) had detectable ctDNA pre-operatively Post-operative results were available for 33 patients, and 3 (9%) had detectable tDNA (of those 2 had Inferior vena cava involvement). The first patient developed metastatic disease. The two other patients are receiving adjuvant immunotherapy. Analysis of 50 patients with solely malignant RCC revealed that patients with detectable versus undetectable ctDNA were older 67 vs. 54 years (p=0.03), had a higher pathological stage (p= 0.002), larger tumors (7.2 vs. 4.7 cm, p = 0.004), and higher pathological grade (grade 3-4 vs. grade 1-2; p=0.035) (Table 1). All the patients with renal vein or inferior vena cava involvement had detectable ctDNA (n=8). Conclusions: In our cohort, preoperative ctDNA was detectable in 50% of patients with suspected clinically localized RCC. Detectable ctDNA preoperatively correlated with clinically relevant features. The ability of preoperative ctDNA to predict recurrence and survival in patients with clinically localized RCC warrants further evaluation. [Table: see text]

Hepatocellular Carcinoma with Inferior Vena Cava Thrombus in a Child

Hepatocellular carcinoma (HCC) in children is the second common malignant liver tumor after hepatoblastoma. Chronic hepatitis B virus (HBV) infection causes most hepatocellular cancer worldwide. Metastases to the inferior vena cava (IVC) and right atrium (RA) tumor thrombi are even less common. We reported a case of a 13-year-8-month-old girl with HCC and IVC involvement. Vascular invasion predicts poor overall survival in HCC patients. Patients with HCC involving the IVC had a higher risk of sudden mortality and a poor treatment outcome. It is difficult to treat, and no standard therapy has been established. This case report aimed to describe HCC with IVC involvement. This is a rare disease with a poor prognosis, therefore it is important to detect and manage early.

Vascular pathology in patients with alveolar echinococcosis: framework for assessment and clinical management – a retrospective case series

ObjectiveAlveolar echinococcosis (AE) is a parasitic liver disease with infiltrative growth similar to solid organ malignancies. Major vascular damage is frequent and often remains untreated until catastrophic events precipitate. Detailed...

Future liver remnant augmentation preceding ex vivo hepatectomy with IVC replacement: a strategy to achieve R0 margins.

Ex vivo hepatectomy with autotransplantation (EHAT) provides opportunity for R0 resection. As EHAT outcomes after future liver remnant (FLR) augmentation techniques are not well documented, we examine results of EHAT after augmentation for malignant tumors. Retrospective analysis of six cases of EHAT was performed. Of these, four occurred after preoperative FLR augmentation between 2018 and 2022. Six patients were offered EHAT of 26 potential candidates. Indications for resection were involvement of hepatic vein outflow and inferior vena cava (IVC) with metastatic colorectal carcinoma (n = 3), cholangiocarcinoma (n = 2), or leiomyosarcoma (n = 1). Five patients were treated with neoadjuvant chemotherapy and four had preoperative liver augmentation. One hundred percent of cases achieved R0 resection. Of the augmented cases, three patients are alive after median follow-up of 28 months. Postoperative mortality due to liver failure was 25% (n = 1). For select patients with locally advanced tumors involving all hepatic veins and the IVC for whom conventional resection is not an option, EHAT provides opportunity for R0 resection. In addition, in patients with inadequate FLR volume, further operative candidacy with acceptable results can be achieved by combined liver augmentation techniques. To better characterize outcomes in this small subset, a registry is needed.

Preprocedural imaging modalities in patients undergoing iliocaval venous recanalization and stent placement.

To determine the diagnostic accuracy of preinterventional imaging modalities in patients being evaluated for iliocaval venous recanalization and stent placement. Consecutive patients with iliocaval postthrombotic obstructions or nonthrombotic iliac vein lesions (NIVL), who were scheduled for recanalization, underwent duplex ultrasound (DUS), magnetic resonance venography (MRV), multiplanar venography (MPV), and intravascular ultrasound (IVUS). The diagnostic accuracies of DUS, MRV, and MPV were analyzed using IVUS as reference. A total of 216 limbs in 108 patients (80 patients with postthrombotic obstructions, 28 patients with NIVL) were examined. In patients with postthrombotic obstructions, the diagnostic sensitivities for the detection of lesions of the common femoral vein were 81% (95% CI 71-89%) for DUS, 76% (95% CI 65-85%) for MRV, and 86% (95% CI 76-93%) for MPV. The sensitivities for detecting lesions of the iliac veins were 96% (95% CI 89-99%) for DUS, 99% (95% CI 92-100%) for MRV, and 100% (95% CI 94-100%) for MPV. Regarding the inferior vena cava, the sensitivities were 44% (95% CI 24-65%) for DUS, 52% (95% CI 31-73%) for MRV, and 70% (95% CI 47-86%) for MPV. The sensitivities for detecting NIVL were 58% (95% CI 34-79%) for DUS, 90% (95% CI 68-97%) for MRV, and 95% (95% CI 73-99%) for MPV. In patients scheduled for recanalization of iliocaval postthrombotic obstructions, the sensitivities of DUS, MRV, and MPV were similar. In patients with suspected inferior vena cava involvement and in patients with NIVL, additional imaging with MR or conventional venography is required.

Renal cell carcinoma T staging: Diagnostic accuracy of preoperative contrast-enhanced computed tomography.

Renal cell carcinoma (RCC) accounts for 1-2% of all malignancies and is the most common renal tumor in adults. Imaging studies are used for diagnosis and staging. Tumor-Node-Metastasis staging strongly affects prognosis and management, while contrast-enhanced computed tomography (CECT) is regarded as a standard imaging technique for local and distant staging. The present study aimed to evaluate the accuracy of CECT for the preoperative staging of RCC by using surgical and pathological staging as the reference methods. This single-center prospective study was conducted between October 2019 and November 2021. The preoperative abdominal CT scans of patients suspected of having RCC were reviewed. Imaging data were collected, including tumor side and size, and perinephric fat invasion. Intraoperative notes were recorded, including the operation type, perinephric fat invasion, renal vein (RV) or inferior vena cava (IVC) tumor extension, and surrounding organ invasion. pathological data were collected on tumor size, RCC type, presence of clear margins, presence of renal capsule or perinephric fat invasion, renal sinus or pelvicalyceal system (PCS) invasion, segmental or main RV extension, and the involvement of Gerota's fascia and nearby organs. Preoperative CECT revealed that 42 out of 59 tumors had a greater maximum diameter than the pathological specimen, with an overall disparity of 0.25 cm. The specificity of CT for the detection of tumor invasion of the perinephric and renal sinus fat and PCS was 95%, and the sensitivity ranged from 80 to 88%. CT had an 83% sensitivity and a 95 specificity in detecting T4 stage cancer, with a 100% specificity for adrenal invasion. The concordance between radiographic and histological results for RV and IVC involvement was high, with specificities of 94 and 98%, and sensitivities of 80 and 100%, respectively. Overall accuracy for correct T staging was 80%. In conclusion, CECT is accurate in the local T staging of RCC, with high sensitivity and specificity for estimating tumor size and detecting extension to nearby structures and venous invasion.

Association Between Inferior Vena Cava-Involved Thrombosis, Severe Iliac Vein Stenosis, and Proximal Pulmonary Embolism in Patients With Deep Vein Thrombosis.

To investigate the possible risk factors for proximal pulmonary embolism (PE) in patients with lower extremity deep vein thrombosis (LEDVT). A single-center, retrospective cohort study was conducted. Consecutive patients diagnosed with unilateral LEDVT and confirmed PE by computed tomography (CT) pulmonary angiography and indirect CT venography between September 2015 and January 2022 were included. Patient demographics, presentation of LEDVT, laboratory examination, thrombus characteristics, comorbidities and risk factors for LEDVT, and imaging findings were analyzed to determine the univariable and multivariable associations. The associations between thrombus location, stenosis, and proximal PE were assessed using a stratified analysis. A total of 115 eligible patients (60.08 ± 15.18 years, 59.1% male) were included. Of these patients, 40 (34.8%) were in the proximal PE group (proximal thrombus involved main or lobar pulmonary artery), and 75 (65.2%) were in the distal PE group (without main and labor pulmonary artery involvement). The univariable analysis showed that onset time of LEDVT symptoms ≤ 7 days (P = .041), inferior vena cava (IVC) involvement (P = .035), and severe iliac vein stenosis (IVS) (stenosis ratio > 61.3%) (P = .010) were associated with the occurrence of proximal PE. Further multivariant analysis showed that IVC involvement was associated with an increased odds of proximal PE (OR: 4.45, 95% CI: 1.083 - 18.248, P = .038), whereas severe IVS (stenosis ratio > 61.3%) was associated with a decreased odds of proximal PE (OR: 0.294, 95% CI: 0.110-0.783, P = .014). Among patients with unilateral LEDVT, IVC involvement increased the risk of proximal PE; patients with severe IVS (stenosis ratio > 61.3%) had a lower risk of developing proximal PE compared with distal PE; and severe IVS seemed to be a protective factor for proximal PE.

Radiotherapy with Multimodality Therapy for Hepatocellular Carcinoma with Hepatic Vein and/or Inferior Vena Cava Tumor Thrombus

<h3>Purpose/Objective(s)</h3> Liver cancer is the seventh most common malignancy and second leading cause of cancer-related death worldwide. Hepatic vein and/or inferior vena cava (IVC) tumor thrombus are less common. Improvement in techniques allowed the increasing use of radiotherapy in treating tumor thrombus in hepatocellular carcinoma (HCC). Hence, the present study evaluated the treatment response and survival outcomes of HCC with hepatic vein tumor thrombus (HVTT) and/or IVC involvement receiving radiotherapy in modern RT technique combined with other multimodality therapies. <h3>Materials/Methods</h3> HCC patients with HVTT and/or IVC tumor thrombus receiving radiotherapy were identified at our institution. All patients were treated with VMAT or IMRT technique and 4D-CT was used in simulation. All thrombus and primary tumor in liver were included in the GTV unless they were far from gross primary tumor. The prescription dose to 95% of the PTV was planned at 50-65Gy in 22-28 fractions over 5-6 weeks. After the completion of RT, chest-abdominal CT and liver MRI were performed at 1, 3, 6, 12 months and then 3 to 6 months thereafter. Treatment response was defined as the best response evaluation in 3 months after RT. Overall survival (OS), progression free survival (PFS) and in-field PFS were recorded during follow-up. <h3>Results</h3> Between April 2010 and September 2021, 30 men and 4 women with a median age of 53.5 years (range 30-71 years) were retrospectively included in the cohort. 7 (20.6%) patients had lymph node metastasis and 10 (29.4%) patients had distant metastasis before radiotherapy (RT). In addition to hepatic vein/inferior vena cava (IVC) tumor thrombus, portal vein and right atrium involvement were present in 17 (50.0%) and 6 (17.6%) patients. Half of the patients received TACE and 14 (41.2%) patients received systemic treatment including target therapy and immune therapy before radiotherapy. Most patients received target therapy concurrent with (70.6%) and/or post RT (79.4%). During follow-up (median 13.0 months), the median PFS and median in-field PFS were 4.2 months and not reached, respectively. The PFS and in-field PFS rate were 24.6% and 79.2% at 1 year, 19.7% and 72.0% at 2 years. The OS rate was 77.6% at 1 year and 36.3% at 2 years with a median OS of 15.8 months. PFS and OS were not associated with portal vein involvement, IVC involvement and distant metastasis before RT. The most common first failure site was lung (13/34 patients, 38.2%), followed by liver (7/34 patients, 20.6%). Only 5 (14.7%) patients had progression in the RT field of liver during follow-up. No patients had radiation-induced liver disease or pulmonary embolism during follow-up. <h3>Conclusion</h3> Our study proved that HCC lesions and tumor thrombus were sensitive to RT even after other local therapies. Combination of RT and systemic therapy in treating HCC patients with HVTT and IVC tumor thrombus was safe and effective.

Right and left atrial metastasis of renal cell carcinoma: A case report

Introduction and importanceCardiac tumors are uncommon with an estimated incidence of 0.002–0.3 % in autopsy series. Most cardiac tumors are metastatic in nature. Renal cell carcinoma (RCC) metastatic to the heart without inferior vena cava (IVC) contiguous involvement is extremely rare with about 31 cases reported in the literature and only one case with bilateral atrial metastases.Case presentationIn this report, the surgical management of metachronous RCC involving the right and left atrium is described in a 41-year-old male patient three years after initial diagnosis who presented with worsening episodes of cough, dyspnea, chest pain and hemoptysis. Transesophageal echocardiogram revealed significant inflow obstruction. The patient underwent bilateral atrial mass excision via median sternotomy. The postoperative period was unremarkable, and the patient was referred to medical oncology to pursue further treatment.Clinical discussionAmong the reported cases of cardiac RCC metastases without contiguous IVC involvement, bilateral atrial metastases are exceedingly rare. To our knowledge, this is the first case with bilateral atrial involvement to undergo surgical resection reported in the literature.ConclusionIsolated biatrial cardiac metastases from RCC can be successfully resected with good outcomes in selected patients.

Primitive neuroectodermal tumor of the kidney: an experience of three cases

BackgroundPrimitive neuroectodermal tumors (PNET) are highly aggressive tumors belonging to the Ewing’s sarcoma family. Primitive neuroectodermal tumor is a group of bone and soft tissue tumor arising from neuroectodermal tissues. They are characterized by the expression of MIC2 and neural markers like neuron-specific enolase, synaptophysin, S-100, vimentin, Leu-7, and the presence of the EWS-FLI1 translocation. It is a rare malignancy with only about 150 cases being published worldwide. The tumor usually presents with non-specific symptoms such as abdomen pain and hematuria. Inferior vena cava (IVC) involvement by renal PNET is extremely rare with only a handful cases reported in the literature and is a clinically challenging scenario. There is a dearth of data on the management of this disease; further, the management of this disease in current scenarios is not well reported. We report our experience with three cases over 11 years which spans all three spectrums of the disease: localized, locally advance and metastasis. Case presentationCase 1: 37-year-old gentleman presenting with hematuria found to have right renal tumor with IVC invasion which was found to be unresectable intraoperatively. Patient was started on chemotherapy but deteriorated and died 3 months later. Case 2: 38-year-old gentleman presenting with abdominal pain found to have locally advanced left renal tumor. Patient developed local recurrence and metastasis 15 months after resection but had stable disease for nearly 4 years before developing acute myeloid leukemia and withdrawing consent for treatment. Case 3: 13-year-old girl who presented with right abdominal mass and subsequently found to have localized disease. After aggressive chemotherapy, patient is now recurrence-free after nearly 11 years.ConclusionsOur study aims to show the spectrum of renal primitive neuroectodermal tumors with outcomes in all three clinical scenarios, with the aim of reinforcing that high index of suspicion for this disease is required in young patients presenting with aggressive disease, and immunohistochemistry should be used when in doubt, as outcomes are greatly improved with adjuvant chemotherapy.

Resection of retroperitoneal tumors with inferior vena cava involvement without caval reconstruction.

Retroperitoneal tumors with involvement of the inferior vena cava (IVC) often require resection of the IVC to achieve complete tumor removal. This study evaluates the safety and efficacy of IVC ligation without caval reconstruction. A retrospective chart review of patients who underwent IVC ligation(IVC-Ligation) and IVC resection with reconstruction (IVC-Reconstruction) at our institution between May 2004 and April 2021 was performed. Outcomes from the two surgical techniques were compared via univariate analysis using the Kruskal-Wallis test for continuous variables and Fisher's exact test for categorical variables. Forty-nineIVC-Ligation and sixIVC-Reconstruction surgeries were identified. There were no differences in baseline demographics, tumor characteristics, complication rates, postoperative morbidity, or overall 5-year survival between groups. IVC-Reconstruction patients were more likely to require intensive care unit admission (83% vs. 33%; p = 0.0257) and the IVC-Ligation cohort had a tendency to present with nondebilitating postoperative lymphedema (35% vs. 0%; p = 0.1615), which resolved for most patients. IVC-Ligation is a viable surgical option for select patients presenting with retroperitoneal tumors with IVC involvement and provides acceptable short- and medium-term outcomes.

Inferior vena cava involvement in renal cell carcinoma: if you fail to plan, you're planning to fail.

Inferior vena cava involvement in renal cell carcinoma: if you fail to plan, you're planning to fail.

Silent Pulmonary Embolism in Deep Vein Thrombosis: Relationship and Risk Factors.

PurposeThis study aimed to evaluate risk factors for silent pulmonary embolism (PE) in symptomatic deep vein thrombosis (DVT) and investigate the relationship between DVT and silent PE.MethodsThis was a single-centre, retrospective cohort study. Between 5 January 2015 and 31 December 2021, consecutive patients with symptomatic DVT received CT pulmonary angiography and CT venography were analyzed. Patient demographics, comorbidities, risk factors, and image findings were analyzed. The group differences were compared using a Chi-square test, Fisher's exact test, independent t test, or Mann-Whitney U test. Multivariant regression was used to determine predictive factors for silent PE.ResultsA total of 355 patients (mean age, 60.5 ± 16.6 years) were included. The incidence of silent PE was 43.1%. The main or lobar pulmonary arteries were affected in 53.6% of patients, which is more often found in iliofemoral DVTs (56.6% vs 26.7%, p = .027). The multivariant analysis showed male patients (p = .042; OR 1.59; 95% CI, 1.02–2.50), inferior vena cava involvement (p = .043; OR 1.81; 95% CI, 1.02–3.20) and D-dimer value > 3.82 μg/ml (p < .001; OR 2.32; 95% CI, 1.43–3.77) were risk factors for silent PE. Unilateral DVT patients with ipsilateral iliac vein compression had a lower incidence of silent PE (28.8% vs 52.9%, p < .001).ConclusionIliofemoral DVT was associated with a more proximal PE. The male patients, inferior vena cava involvement, and D-dimer > 3.82 μg/ml were risk factors for silent PE. Ipsilateral iliac vein compression reduced the incidence of silent PE.

SURGICAL TECHNIQUE IN LEIOMYOSARCOMA OF THE INFERIOR VENA CAVA DEPENDING ON ITS LOCATION

The aim: To improve the outcomes of inferior vena cava (IVC) leiomyosarcoma, propose own classification of IVC segments, which correlates with surgical access, methodology, sequence and amount of surgery. Materials and methods: In the period from 1991 to 2021 in the Transcarpathian Regional Clinical Hospital named after A. Novak and in the Transcarpathian Antitumor Center 8 patients with IVC leiomyosarcoma were operated. The prevalence of leiomyosarcoma in IVC was determined according to the division of IVC into 7 segments. Defeat of one segment of IVC was in 50% of cases, two - in 37.5%, three - in 12.5%. In 5 (62.5%) cases circular resection and alloprosthesis of IVC were performed; in 2 (25%) – circular resection, alloprosthesis of IVC and implantation of the right and left renal veins in the prosthesis; in 1 (12.5%) - circular resection, alloprosthesis of IVC and implantation of the left renal vein in the prosthesis. All surgeries were performed with laparotomy access (87.5% by Chevron type). Results: The average operation time was 215 (160-320) minutes, the average blood loss was 305 (250-500) ml. Postoperative complications were recorded in 2 (25%) cases. There were no cases of pulmonary embolism, venous thrombosis, prosthesis thrombosis, perioperative mortality. In 7 (87.5%) cases, surgery was radical. The overall 1-year, 2-year and 3-year survival rates were 87.5%, 71.4% and 57.7%. Conclusions: The division of IVC into 7 segments characterizes the detailed definition of the cranial limit of leiomyosarcoma and segmental involvement of IVC in the tumor process, which allows to choose the right surgical tactics, perform radical resection of IVC and maintain laminar blood flow to IVC and its tributaries.

SURGICAL TECHNIQUE IN LEIOMYOSARCOMA OF THE INFERIOR VENA CAVA DEPENDING ON ITS LOCATION.

The aim: To improve the outcomes of inferior vena cava (IVC) leiomyosarcoma, propose own classification of IVC segments, which correlates with surgical access, methodology, sequence and amount of surgery. Materials and methods: In the period from 1991 to 2021 in the Transcarpathian Regional Clinical Hospital named after A. Novak and in the Transcarpathian Antitumor Center 8 patients with IVC leiomyosarcoma were operated. The prevalence of leiomyosarcoma in IVC was determined according to the division of IVC into 7 segments. Defeat of one segment of IVC was in 50% of cases, two - in 37.5%, three - in 12.5%. In 5 (62.5%) cases circular resection and alloprosthesis of IVC were performed; in 2 (25%) - circular resection, alloprosthesis of IVC and implantation of the right and left renal veins in the prosthesis; in 1 (12.5%) - circular resection, alloprosthesis of IVC and implantation of the left renal vein in the prosthesis. All surgeries were performed with laparotomy access (87.5% by Chevron type). Results: The average operation time was 215 (160-320) minutes, the average blood loss was 305 (250-500) ml. Postoperative complications were recorded in 2 (25%) cases. There were no cases of pulmonary embolism, venous thrombosis, prosthesis thrombosis, perioperative mortality. In 7 (87.5%) cases, surgery was radical. The overall 1-year, 2-year and 3-year survival rates were 87.5%, 71.4% and 57.7%. Conclusions: The division of IVC into 7 segments characterizes the detailed definition of the cranial limit of leiomyosarcoma and segmental involvement of IVC in the tumor process, which allows to choose the right surgical tactics, perform radical resection of IVC and maintain laminar blood flow to IVC and its tributaries.

Metastatic Adrenocortical Carcinoma Presenting as Intracavitary Mass in Right Atrium: A Case Report

1.1. Background: Adrenocortical carcinomas grow rapidly and tend to metastasize to liver and lungs and invade kidney, renal vein and Inferior vena cava (IVC). Cardiac metastases of the tumor is rare, less than 20 cases have been described and most of them occurred via direct extension through inferior vena cava into right atrium [1]. Endocardial Metastases from adrenocortical carcinoma presenting as an intracavitary growth in right atrium of heart without associated IVC involvement is very rare. 1.2. Case Presentation: We describe a case of 20 years old young male who presented to emergency with gradual worsening of dyspnea and facial swelling for last 6 months. Echocardiography showed a large Right atrial mass filling the whole chamber, attached to IAS (interatrial septum) via thin stalk suggestive of right atrial myxoma. Patient underwent resection of myxoma and histopathology proved it to be metastatic adrenocortical neoplasm. Whole body contrast enhanced CT scan showed primary large soft tissue mass lesion in right suprarenal location with metastatic bilateral lung metastases. On biochemical investigation associated hypercortisolemia was found. 1.3. Conclusion: Usually renal cell carcinomas spread intraluminally via inferior vena cava into the right atrium (reported in 1 % of tumors). Intracavitary presentation of metastatic adrenocortical carcinoma is very rare, and index case report was a similar presentation of a young male that underwent resection of intracavitary growth of right atrium (suspected as Myxoma), that came out to be metastatic adrenocortical carcinoma. In patients with advanced local or metastatic adrenocortical carcinomas, not amenable to surgical resection, cytotoxic chemotherapy may be attempted.

A systematic review of the current management approaches in leiomyosarcoma of inferior vena cava-Results from analysis of 118 cases.

Primary intravenous leiomyosarcomas are rare vascular tumors with aggressive disease biology. The diagnosis and management have been challenging as little data exist from large databases. A literature search was done to identify all cases of primary leiomyosarcomas in the last five years. Clinicopathological features and management strategies were evaluated. The median age was 53 years, predominantly females (2.5:1), presenting as metastases in up to 12.1% cases. Most tumors were locally advanced with a median size of 10cm. Inferior vena cava involvement from renal veins to infrahepatic veins remains the most frequent site (57.1%cases) while nearly half (52.8%) proceeded for surgery without histological proof. Most patients could undergo upfront resection (88.0%) with few patients receiving neoadjuvant chemotherapy (4.3%) or neoadjuvant radiotherapy (2.2%). Significant multivisceral resections included right nephrectomy (41.3%), liver resection (25.7%) and left nephrectomy (2.2%). Most patients (91.8%) needed an inferior vena cava graft placement with remarkable microscopically negative margins (85.5% cases). Doxorubicin and ifosfamide were the most frequently used combination chemotherapy regimens in both pre and postoperative settings with partial responses. The median overall and disease free survival among operated patients was 60 months and 28 months respectively. In multivariate analysis large tumor, extensive inferior vena cava involvement, and need for adjuvant chemotherapy appeared significant predictors for overall survival. Aggressive upfront surgical resection with clear margin remains the key for long-term survival. Doxorubicin-based regimens were preferred as neoadjuvant chemotherapy while adjuvant treatment with chemotherapy, radiotherapy, or both may be considered in high-risk patients.

Budd-Chiari syndrome in Behçet's disease: a retrospective multicenter study.

To compare the clinical features, laboratory findings, and prognosis of Behçet's disease (BD) patients with and without Budd-Chiari syndrome (BCS). This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14-47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points • Mortality rate is higher in BD-associated BCS patients with IVC involvement. • Chronic and silent form of BD-associated BCS has a better prognosis. • The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial.