Background: Renal angiomyolipoma (AML) without local invasion is generally considered benign. However, it may extend to the renal sinus, even the renal vein, or the inferior vena cava (IVC). In patients with non-tuberous sclerosis complex, coexistence of renal cell carcinoma (RCC) and renal AML is uncommon. Case presentation: A 72-year-old woman was incidentally found to have a solitary right renal mass with an IVC thrombus extending into the right atrium during a routine health checkup. Robot-assisted laparoscopic radical nephrectomy and thrombectomy were successfully performed through adequate preoperative examination and preparation. Two tumor lesions were found and pathologically confirmed as renal AML and RCC, and the tumor thrombus was derived from the renal AML. During the one-year follow-up period, no signs of recurrence or metastatic disease were observed. Conclusions: Renal AML with a tumor thrombus in the IVC and right atrium accompanied by RCC may occur, although rarely. In clinical practice, if preoperative manifestations differ from those of common diseases, rare diseases must be considered to avoid missed diagnoses. In addition, adequate examination and multidisciplinary discussions before making a diagnosis are necessary. For a level 4 tumor thrombus with no infringement of the venous wall, adoption of robot-assisted minimally invasive surgery, without extracorporeal circulation technology, is feasible.

UNASSIGNED: LMS of IVC needs a multidisciplinary approach. Surgical excision with free margin is the cornerstone of management. Upon case-by-case selection, adjuvant chemotherapy may play a role in better oncologic outcome.
UNASSIGNED: Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare form of mesenchymal origin malignancy with less than 400 cases reported to date. Surgery is the mainstay of management but it requires vast experience in vascular and visceral surgery to attain a free tumor margin. Subsequent adjuvant treatment with chemotherapy and radiation remains as an area of gray zone. We report the case of a 61-year-old man with an 8-month history of abdominal pain. Upon physical examination, an ill-defined mass over the right side of the lower abdomen and bilateral lower extremity edema were detected. Abdominal ultrasound with Doppler revealed a right-side retroperitoneal mass invading the IVC with extensive venous thrombosis for which anticoagulation was initiated. Computed Tomography of the abdomen revealed a huge heterogeneously enhancing mass involving the whole length of the infrarenal IVC obstructing the IVC lumen with collateral veins draining through the paralumbar veins and inferior epigastric veins bilaterally. With a top differential of primary IVC LMS, a midline longitudinal laparotomy was performed with an intraoperative finding of a tumor arising from the infra-renal IVC which was excised. Gore-Tex graft was used to reconstruct the IVC. There was an injury to the right common iliac artery and it was repaired by end-to-end anastomosis. Histopathology confirmed a high-grade LMS of the IVC and surgical margin status was unknown. He was given adjuvant Chemotherapy consisting of Doxorubicin and Dacarbazine. He has been on follow-up at the Oncology side with a good performance status.

Ewing sarcoma (ES) is a rare group of undifferentiated tumors that originate from neuroectoderm. Although the overall prognosis is poor, early diagnosis and treatment by a multidisciplinary team with multimodal therapy can improve outcomes. Therefore, we present a 22-year-old female patient with primary renal ES with tumor thrombosis up to the vena cava who had radical nephrectomy and IVC tumor thrombectomy followed by adjuvant chemotherapy because a preoperative percutaneous biopsy was confirmed the diagnosis.

Agenesis of the inferior vena cava (IVC) is a rare congenital anomaly that is associated with the development of extensive collateral circulation with the aim of compensating for the inadequate return of blood to the right ventricle. This collateral circulation predisposes to the emergence of venous hypertension with stasis and thrombus formation. Most cases are asymptomatic and are diagnosed incidentally. We report the case of a 28-year-old man who presented with bilateral deep vein thrombosis (DVT) as the first manifestation of agenesis of the IVC. We decided to maintain anticoagulation for an indefinite period of time after a multidisciplinary discussion. IVC agenesis should be considered a cause of DVT in young men, with bilateral and proximal thrombosis and without other risk factors. The rarity of the condition makes its therapeutic approach complex.

Nephrostomy catheter misplacement into the inferior vena cava after percutaneous nephrolithotomy is an extremely rare complication, and subsequent catheter-related thrombosis has been more rarely reported. Here, we report a rare case of nephrostomy catheter misplacement after percutaneous nephrolithotomy. During the procedure, due to bleeding upon establishing the puncture channel, a renal fistula catheter with a balloon was inserted to facilitate hemostasis. However, the catheter inadvertently migrated into the inferior vena cava, with the inflated balloon obstructing venous return, resulting in thrombosis formation within the inferior vena cava. The patient was urgently transferred to our hospital for intervention. Upon administering anticoagulation and antimicrobial therapy, we first placed a filter in the patient\'s inferior vena cava to prevent thrombus embolism to the pulmonary arteries during catheter removal. Under fluoroscopy, the catheter was withdrawn into the renal vein, followed by catheter-directed thrombolysis and thrombus aspiration. Eventually, the renal fistula catheter was gradually removed in stages without any bleeding and pulmonary embolism occurring throughout the treatment process. Through a review of relevant literatures, we analyzed the reasons for catheter misplacement and summarized the associated treatment experience.

Polysplenia syndrome is an embryological disorder whereby the usual left-right asymmetry of thoracic and abdominal viscera fails to develop. It is a rare entity, estimated to occur at a frequency of 1 in 40,000, and is often associated with cardiac and biliary abnormalities. More than 75% of patients die before the age of 5 years, and even in the absence of cardiac anomalies, only 5%-10% of patients are expected to survive into adulthood without complications. Although polysplenia syndrome encompasses a wide range of anatomic abnormalities, there is no single pathognomonic feature. Hence, the prognosis of patients with polysplenia depends on their anatomy, thus necessitating radiology in their management. Here we present a case of a 56-year-old man with polysplenia syndrome and situs inversus totalis. This presentation is atypical because polysplenia is usually considered a form of situs ambiguus, and cases with situs inversus totalis are exceedingly rare. Also noted in our patient are variations in the great vessels, including aortic arch branches and the venae cavae which are features not typically associated with either polysplenia syndrome or situs inversus totalis. The patient is healthy and asymptomatic at baseline, with his diagnosis being made incidentally. Our case report is the first to describe this unique combination of cardiothoracic and cardiovascular anatomy. It also emphasizes the importance of radiologists in caring for patients with laterality defects. As these disorders are uncommon, more data on their anatomic variations may help provide better medical care to this patient population.

Abnormal traffic between the left atrium (LA) and inferior vena cava (IVC) in the database is currently rare. Herein, we present a unique case of abnormal traffic between the LA and the IVC, which was diagnosed using transesophageal echocardiography and confirmed by computed tomography angiography. This case substantiates the superiority of transesophageal echocardiography over transthoracic echocardiography in detecting specific site lesions.

UNASSIGNED: Anomalous drainage of inferior vena cava (IVC) into left atrium (LA) is a rare aetiology of cyanosis in adults. This condition may be associated with atrial septal defects, anomalous pulmonary venous drainage, and pulmonary arteriovenous fistulas. This case report presents an instance of anomalous drainage of IVC into LA, associated with ostium secundum atrial septal defect (ASD). It contributes to the existing literature by highlighting the diagnostic challenges associated with this anomaly, particularly during surgical intervention.
UNASSIGNED: A 38-year-old male presented with a six-year history of exertional dyspnoea and episodic palpitation. Transthoracic echocardiography revealed a large secundum (ASD), the IVC draining into LA, a left superior vena cava, and mild mitral regurgitation. These findings were further confirmed by right and left heart catheterization and CT angiogram. The patient was referred to CTVS department for surgical correction. The post-operative course was uneventful. At a 1.4-year follow-up, the patient reported significant improvement in symptoms.
UNASSIGNED: We present a case of anomalous drainage of IVC into LA, associated with ostium secundum ASD. An ASD co-occurs with this condition in ∼70% of the reported cases. This anomaly differs from a low or inferior vena caval secundum ASD, where a prominent Eustachian valve can cause blood shunting from the IVC to LA. If the surgeon is not vigilant, this can be mistaken for the inferior ASD rim, potentially leading to iatrogenic diversion of IVC blood to LA upon ASD closure, resulting in cyanosis. This case underscores the diagnostic and surgical challenges associated with this condition.

Tumors of the inferior vena cava (IVC) are rare and usually malignant and they can be primary and secondary. The most common primary tumor of the IVC is primary leiomyosarcoma. The first case of primary IVC leiomyosarcoma has been described in 1871 [1].The total number of 218 cases has collected until 1996 [2]. After that, three large single center series of these tumors emerged [3-5]. Present a series of five cases of these tumors. All the patients underwent a wide complete resection of tumors and the reconstruction with Dacron grafts. One patient died 19 months after the surgery, while the remaining ones survived without a local and system disease relapse. Although a surgical resection combined with the chemotherapy is often not curative, it can achieve a significant long-term survival. For this reason, we recommend the aggressive surgical management using the modern vascular surgical and oncology techniques.

Agenesis of inferior vena cava (AIVC) is an extremely rare congenital abnormality. In AIVC, venous flow from the lower extremities enter superior vena cava mainly through the azygous and hemiazygous system, forming anastomotic collateral vessels. A global increase in intra-abdominal pressure by the gravid uterus may further stress the collateral system, increase venous stasis and decrease venous return. We present the management of a 37-year old pregnant woman with AIVC who underwent caesarean section with norepinephrine infusion and general anaesthesia. She presented with shortness of breath when seated, episodes of dizziness while walking or sitting upright with subsequent tachycardia. Cardiac status was monitored using an arterial pulse contour CO monitor. We did not observe large fluctuations in CO, SV, MAP during induction and intubation as well as during delivery. We believe that administration of an infusion of norepinephrine from induction to anaesthesia through caesarean section contributed to this result. Sympathetic activation caused venoconstriction, which significantly increased venous return and maintained haemodynamic stability.