Intracardiac Tumor Research Articles

Surgical management and outcomes of renal tumors with inferior vena cava extension among children: a single center retrospective study from Pakistan

ObjectiveThe aim of this study was to assess management and determine outcomes of renal tumors with inferior vena cava (IVC) and intracardiac (IC) extension in a tertiary care center in Pakistan.MethodsA retrospective chart review was conducted at the Aga Khan University Hospital, Karachi, Pakistan. All patients from 1 to 18 years of age with renal tumors with intravascular extensions, surgically managed from January 1988 till June 2016, were included. Data was extracted by reviewing medical records, and the tumor details, treatment and outcomes were analyzed.ResultsA total of 18 patients out of the total 61 patients with renal tumors, presented with IVC and/or IC extension, with the majority involving the right kidney. Mean age was 5.9 (SD:4.9) and a female preponderance (56%) was seen. Wilms tumor (77%) was the most common tumor type, with the level of tumor extension into IVC predominantly being below the diaphragm (55.5%). Fourteen patients received preoperative chemotherapy, with tumor regression, seen in 10. Most patients underwent thrombectomy through the renal vein (56%). Regarding outcomes, frequency of mortality and morbidity was 1 and 2, respectively, with 7 patients having no recurrent 5 years post-surgery.ConclusionA greater incidence (29.5%) of IVC and or IC Tumor extension was found compared to existing literature, which could likely be due to a higher referral rate to the center. Moreover, this is a single-center study and so a multi-center study is crucial to form an assessment of surgical management in resource-limited settings. Our study is the first from Pakistan on this particular renal tumor presentation. Considering the varying case presentations and surgical techniques used, further studies are needed to standardize surgical management and optimize patient outcomes.

Pilot study to assess the early cardiac safety of carbon ion radiotherapy for intra- and para-cardiac tumours.

Modern photon radiotherapy effectively spares cardiac structures more than previous volumetric approaches. Still, it is related to non-negligible cardiac toxicity due to the low-dose bath of surrounding normal tissues. However, the dosimetric advantages of particle radiotherapy make it apromising treatment for para- and intra-cardiac tumours. In the current short report, we evaluate the cardiac safety profile of carbon ion radiotherapy (CIRT) for radioresistant intra- and para-cardiac malignancies in areal-world setting. We retrospectively analysed serum biomarkers (TnI, CRP and NT-proBNP), echocardiographic, and both 12-lead and 24-hour Holter electrocardiogram (ECG) data of consecutive patients with radioresistant intra- and para-cardiac tumours irradiated with CIRT between June 2019 and September 2022. In the CIRT planning optimization process, to minimize the delivered doses, we contoured and gave ahigh priority to the cardiac substructures. Weekly re-evaluative 4D computed tomography scans were carried out throughout the treatment. Atotal of 16patients with intra- and para-cardiac localizations of radioresistant tumours were treated up to atotal dose of 70.4 Gy relative biological effectiveness (RBE) and amean heart dose of 2.41 Gy(RBE). We did not record any significant variation of the analysed serum biomarkers after CIRT nor significant changes of echocardiographic features, biventricular strain, or 12-lead and 24-hour Holter ECG parameters during 6months of follow-up. Our pilot study suggests that carbon ion radiotherapy is apromising radiation technique capable of sparing off-target side effects at the cardiac level. Alarger cohort, long-term follow-up and further prospective studies are needed to confirm these findings.

Endovascular thrombectomy provides durable, symptomatic relief in iliocaval tumor thrombosis

Iliocaval venous tumor thrombus is a morbid condition associated with chronic venous stasis, lower extremity edema/pain, extensive thrombus burden and high mortality secondary to critical flow obstruction, intracardiac thrombus extension and tumor embolization. Typically resistant to medical therapy, management is primarily surgical, presenting challenges for medically complex patients with widespread or end-stage disease. Mechanical or aspiration thrombectomy represents an appealing treatment strategy but data are lacking. We performed a single-center, 10-year, retrospective review of patients with pathology-confirmed, iliocaval tumor thrombus who underwent thrombectomy. 14 patients met inclusion criteria and were managed by 18 procedures over this period. The most common malignancy was renal-cell carcinoma (n=7; 50%); other types included germ cell (n=2; 14%), other genitourinary (n=2; 14%), neuroendocrine (n=1; 7%), soft tissue (n=1; 7%), and skin cell malignancies (n=1; 7%). All patients had thrombus involving the distal inferior venous cava (IVC), 50% had bilateral iliac involvement and 29% atrial involvement. Common indications were venous obstruction symptoms (n=11; 65%) and evidence of embolism (n=6; 35%). All patients tolerated the procedures without acute complication. The technical success rate was 94%, with marked improvement of flow and reduction in thrombus burden, and 79% had subjective symptomatic improvement. All patients survived for >2 weeks and 50% had long-term survival of >1 year, with 86% of these patients having renal-cell carcinoma (RCC). Three patients underwent multiple thrombectomies within days to weeks, with ultimate symptomatic improvement. Overall, our study results suggest mechanical or aspiration thrombectomy as a safe and efficacious treatment for patients with iliocaval tumor thrombus.

A Retrospective Analysis of AngioVac Outcomes at a Tertiary Care Center

BackgroundThe AngioVac system is a vacuum aspiration device approved for removal of right-sided cardiac thrombi. It has also been used for management of right-sided endocarditis in selected cases. Retrospective case series have reported high success rate and acceptable 30-day mortality, but there is limited data regarding outcomes beyond the immediate postoperative period. The purpose of this study is to describe our institution's experience with the AngioVac system for thrombus, vegetation, and tumor removal with a significant improvement over previously reported 1-year survival rates. MethodsA retrospective review of AngioVac cases performed at our tertiary care center from 2016-2022 was done. From 2016-2022, 23 patients were identified, and their outcomes are described. ResultsOur review demonstrates 81.8% procedural success, 100% procedure survival, 90.9% survival to discharge, and 81.8% 30-day survival rates. One-year survival rate was 72.7%. Complications including an 18.2% rate of new vasopressor use, 54.5% rate of transfusion requirement, and 4.5% rate of acute renal failure requiring hemodialysis were identified. Intraprocedural embolization occurred in 1 case requiring venoarterial extracorporeal membrane oxygenation support and thrombectomy. One case was converted to open surgical intervention. ConclusionsOur review further supports the safety and efficacy of minimally invasive vacuum-assisted aspiration systems beyond the immediate postoperative period in intracardiac thrombus, tumor, and right-sided infective endocarditis. Our institution's experience emphasizes a team-based approach including interventional cardiology and cardiothoracic surgery with a standardized imaging approach with transesophageal echocardiogram. Future guidelines are needed to include an algorithmic approach to intracardiac masses.

Severe coagulopathy and inflammation occurred after resection of giant right ventricular intimal sarcoma with cardiopulmonary bypass: a case report

BackgroundPrimary malignant cardiac tumors are rare in clinic, and surgical resection under cardiopulmonary bypass (CPB) remains the main treatment. The non-physiological perfusion process of CPB leads to contact activation, and the resulting coagulopathy and systemic inflammatory response syndrome (SIRS) are common complications. However, it is difficult to predict the impact of foreign tumor fragments on this pathophysiological process once they enter the bloodstream, making this phenomenon more complex and challenging.Case PresentationWe report a case of cardiac intimal sarcoma who developed severe coagulopathy and widespread inflammation after excision of massive right ventricular tumor and replacement of tricuspid valve by median sternotomy under CPB. Although the procedure was expected to cause tumor cell necrosis and precautions were taken, uncontrolled massive postoperative bleeding, persistent fever, abnormally elevated inflammatory markers, and recurrent malignant arrhythmias occurred after surgery. In addition to common factors, the most possible underlying mechanism is contact activation triggered following surgical procedure for intimal sarcoma with CPB.ConclusionPatients with intracardiac malignant tumors are at a high risk for serious contact activation during CPB. Preventive application of comprehensive anti-inflammatory measures such as drugs and adsorptive CPB technology, as well as point-of-care (POC) monitoring of coagulation status will be helpful for individualized guidance and optimization of CPB management, and improvement of patient prognosis.

Use of fine-needle puncture under endosonographic control in the diagnosis of left atrium sarcoma – case report

Background. Over the past 30 years, with the development of endoscopic technologies and the advent endoscopic ultrasound-guided fine-needle aspiration (EuS-FnA) it has become possible to diagnose with preliminary verification of cardiac tumors, which in turn is an undoubted advantage in detecting cardiac pathology. A rare clinical case is presented: the detection of a tumor of the left atrium using high-tech endoscopic techniques, in particular a EuS-FnA. when studying the surgical material using immunohistochemistry and FISH hybridization, the final diagnosis sounded like intimal sarcoma. Diagnosis of intimal sarcoma is a complex clinical task, and difficulties in making a diagnosis delay the duration of treatment. Our experience demonstrates the modern role of endoscopy as a minimally invasive method of diagnosing intracardiac tumors.

Primary intracardiac germ cell tumor.

We present the echocardiography images in a 6 year old girl who presented with a history of scalp swelling after trivial trauma which was subsequently diagnosed as metastases from primary intracardiac germ cell tumour.

A novel two-stage approach to the treatment of renal cell carcinoma with intra-cardiac tumour extension and Budd-Chiari syndrome.

To present the early results of a new technique for the treatment of renal cell carcinoma with intra-cardiac tumour extension and Budd-Chiari syndrome. The first stage involves transdiaphragmatic debulking of the right heart, inferior vena cava (IVC) and hepatic veins via median sternotomy, followed by a purse-string suture placed in the IVC below the hepatic veins. The second stage is performed separately and involves en bloc resection of the affected kidney, and IVC and vascular reconstruction via an abdominal incision. Three of five patients presented with clinical Budd-Chiari syndrome; two had radiological features only. The median time between surgical procedures was 12 days (IQR 13 days). Four of the five patients had a R0 resection. While all five patients successfully completed both operative stages, one patient died 22 days after the second stage. Of the remaining four, all survive with no disease recurrence. While we continue to compile longer-term data for a larger follow-up series, these preliminary findings show the feasibility of this technique and support the development of this programme of surgery.

Metastatic Renal Cell Carcinoma Presenting as an Intracardiac Tumour Without Involving the Inferior Vena Cava

Metastatic Renal Cell Carcinoma Presenting as an Intracardiac Tumour Without Involving the Inferior Vena Cava

Abstract 16284: Atypical Cause of Embolic Stroke; Intracardiac Metastasis of Mixed Germ Cell Tumor

Testicular neoplasm constitutes the most common solid organ malignancy in males between the ages of 15 and 35. Metastatic disease to the heart is a rare entity. In most cardiac cases, metastasis is by direct hematogenous spread via the inferior vena cava to the right heart. Metastatic disease to the left heart is even more infrequent. We present the case of intracardiac metastasis of mixed germ cell tumor presenting with an embolic stroke. A 47-year-old Latinx male with a history of stage IV testicular cancer s/p left radical orchiectomy with multiple bilateral pulmonary masses (largest 6x9 cm), atrial fibrillation s/p ablation, left lower extremity iliac thrombus s/p thrombectomy, HTN, and diabetes who presented with sudden onset expressive aphasia and dysarthria. CT head showed a 3x5 cm left occipital intraparenchymal hemorrhage suspected to be an embolic stroke with hemorrhagic conversion. Further evaluation with TTE and TEE revealed a highly mobile and friable multi-lobar mass attached to the anterior mitral leaflet occupying most of the left atrial cavity (4.2x4.9 cm), a small mass attached to the right ventricular aspect of the tricuspid valve, an echo-dense mass in the left lower pulmonary vein at the left atrial junction, and a large mass abutting the rim of the superior vena cava. Definitive surgical management was considered; however, patient was deemed a high-risk candidate given high burden of cardiac metastases. After multidisciplinary discussion, it was decided to pursue systemic chemotherapy. This case illustrates an exceptionally rare presentation of advanced stage testicular cancer metastasizing to the heart with large intracardiac tumor burden resulting in embolic stroke. Early cardiac screening may be beneficial in individuals with history of cancer who present with stroke symptoms. Embolization of cardiac metastasis to other organs poses a great clinical challenge and multidisciplinary decision-making is crucial to clinical management.

Abstract 14737: A Case of an Intracardiac Sarcoma Arising From Metastatic Spread of a Primary Phyllodes Tumor of the Breast

Background: Malignant phyllodes tumors with cardiac involvement are rare, presenting diagnostic and management challenges. The benefits of medical and surgical therapies remain unclear. Large-bore aspiration devices are currently only approved for retrieval of venous thromboemboli but may present a valid option for diagnosis and treatment of intracardiac masses in high risk patients. Case Summary: We present the case of a 57-year-old female with a malignant phyllodes tumor of the breast and bilateral pulmonary emboli who presented with an incidental complex intracardiac mass on transthoracic echocardiography (1). A follow-up transesophageal echocardiogram demonstrated masses within the superior vena cava, right atrium, and right ventricle (2a-2b). Aspiration thrombectomy was performed given clinical stability and concern for thrombi with high risk of worsening clot burden. A large-bore aspiration device was used to retrieve the masses in multiple passes (3), which were pathologically consistent with spindle cell sarcoma. A cardiac MRI was completed following aspiration, and multiple mobile masses persisted in the right ventricle (4). The patient was discharged on day nine of hospitalization with therapeutic chemotherapy planned. She had one session of chemotherapy before a fatal cardiac arrest from unknown cause three months after discharge. Discussion: Phyllodes tumors rarely metastasize to the heart, and effective modalities for their diagnosis and management are unclear. Treatment strategies include wide surgical resection of the primary tumor, however mortality remains high in the setting of metastatic spread. Current guidelines recommend following principles of soft tissue sarcomas with chemotherapy. Large-bore aspiration devices may be an effective way to resect intracardiac tumors for both diagnostic and therapeutic purposes as an alternative to invasive surgical intervention for high-risk patients.

INTRACARDIAC TUMORS: LITERATURE REVIEW OF INTRACARDIAC TUMORS IN ADULTS, BASED ON A CLINICAL CASE OF A LARGE LEFT ATRIAL MYXOMA REVEALED BY CHRONIC PRODUCTIVE COUGH IN A 58-YEAR-OLD WOMAN

We report the clinical case of a 58-year-old woman with chronic productive cough, who allowed us to highlight a tumoral formation in the left atrium suggestive of a myxoma. Based on a literature review, we discuss the chapter on intracardiac tumors.

A primary multiple pleomorphic rhabdomyosarcoma of the heart in an adult dog

BackgroundHeart tumors are rare in dogs. They can be benign or malignant. Clinical signs depend primarily on the location of the tumor and its effect on blood flow.Case presentationAn eleven-year-old crossbreed male dog lethargic and anorectic for previous 3 days was presented to the veterinary clinic. The focused ultrasound assessment with sonograms in trauma (FAST) revealed multiple tumors in the heart which were then confirmed in echocardiographic examination performed by a veterinary cardiologist. Due to the poor general condition and grave prognosis, the dog was humanely euthanized. The autopsy revealed numerous intracardiac tumors in all four heart chambers. No proliferative changes were found in other organs either in thoracic or abdominal cavity. Immunohistochemical examination was performed using formalin-fixed, paraffin-embedded tissue from heart masses. The antibodies against myoglobin, desmin, smooth muscle actin, vimentin, CD34, S100, and pan-cytokeratin (AE1/AE3) were used. Microscopically, the tumor was composed of fascicles of spindle-shaped cells with pale eosinophilic cytoplasm with round, oval, and focally elongated nuclei and one or two prominent nucleoli. The tumor cells showed strong diffuse cytoplasmic immunopositivity for myoglobin and vimentin and focal staining for desmin. Immunostainings for smooth muscle actin-SMA, CD34, pan-cytokeratin, S-100 protein were negative. The immunohistochemical staining pattern confirmed rhabdomyosarcoma.ConclusionsThis is the first description of the primary multiple heart rhabdomyosarcoma in a dog.

Surgical treatment of cardiac tumour thrombus in children.

Cardiac mass in children is rare and insidious onset, and primary cardiac mass is less than secondary mass. Among the malignant tumours in children with tumour thrombus in the venous system, about 98% of the cases are nephroblastoma. But it is still rare for the tumour thrombus to reach the level of the atrium or even enter the right ventricle. In this case, the child complained of chest tightness and palpitation and went to the doctor and found Wilms tumour complicated with intracardiac tumour thrombus.

Thromboembolic diseases from a cardiological point of view

Thromboembolic disease is associated with a high mortality. It can be divided into two groups: embolism from a venous and embolism from an arterial side. This article gives an overview on thromboembolic disease (with a focus on pulmonary embolism and ischemic stroke) from a cardiologist's perspective.The therapeutic options for acute pulmonary embolism range from anticoagulation to fibrinolysis to interventional recanalization and surgery. The deciding factor for choice of therapy is the risk of early death. Besides clinical parameters, laboratory markers like cardiac troponin and right ventricular function on echocardiography or CTPA (computed tomography pulmonary angiography) are used to determine the early mortality risk. In hemodynamically instable patients, immediate thrombolysis is required, whereas patients with intermediate and low risk can be treated with anticoagulation. Interventional recanalization is currently being studied in patients at risk for development of CTEPH (chronic thromboembolic pulmonary hypertension) or an intermediate risk of early mortality.In ischemic stroke, early interdisciplinary workup involving a cardiologist is paramount. Post stroke screening should include monitoring for arrythmias (especially atrial fibrillation) and transthoracic echocardiography as well as sonography of extra- and intracranial arteries. If no embolic source can be detected (embolic stroke of undetermined source), transesophageal echo can be helpful to detect intracardiac shunts like patent foramen ovale (PFO) or intracardiac tumors. Post stroke care includes secondary prevention measures like risk factor modification and lipid lowering therapy as well as anticoagulation. In high risk for paradoxical embolization, interventional PFO closure can be performed. Interventional closure of the left atrial appendage (LAA) can be discussed in patients with both high thromboembolic and bleeding risk.

Usefulness of angioscopy for intracardiac tumour biopsy in a patient with malignant lymphoma.

Usefulness of angioscopy for intracardiac tumour biopsy in a patient with malignant lymphoma.

Minimally Invasive Extirpation of Benign Atrial Cardiac Tumors: Clinical Follow-Up and Survival.

Evidence determining the optimal treatment for cardiac tumors is rare. We report our midterm clinical outcome and patient characteristics of our series undergoing atrial tumor removal through a right lateral minithoracotomy (RLMT). From 2015 to 2021, 51 patients underwent RLMT for atrial tumor extirpation. Patients receiving concomitant atrioventricular valvular, cryoablation, and/or patent foramen ovale closure surgery were included. Follow-up was performed using standardized questionnaires (mean: 1,041 ± 666 days). Follow-up involved any tumor recurrence, clinical symptoms, and any recurrent arterial embolization. Survival analysis was successfully achieved in all patients. Successful surgical resection was achieved in all patients. Mean cardiopulmonary bypass and cross-clamping times were 75 ± 36 and 41 ± 22 min, respectively. The most common tumor location was the left atrium (n = 42, 82.4%). Mean ventilation time was 12.74 ± 17.23 h, intensive care unit stay ranged from 1 to 1.9 days (median: 1 day). Nineteen patients (37.3%) received concomitant surgery. Histopathological analysis showed 38 myxoma (74.5%), 9 papillary fibroelastoma (17.6%), and 4 thrombus (7.8%). Thirty-day mortality was observed in 1 case (2%). One patient (2%) suffered a stroke postoperatively. No patient had a relapse of cardiac tumor. Three patients (9.7%) showed arterial embolization during follow-up. Thirteen follow-up patients (25.5%) were in New York Heart Association class ≤II. Overall survival was 90.2% at 2 years. A minimally invasive approach for benign atrial tumor resection is effective, safe, and reproducible. Of the atrial tumors, 74.5% were myxoma and 82% were located in the left atrium. A low 30-day mortality rate with no manifestation of recurrent intracardiac tumor was observed.

Echocardiography in cardioembolic stroke prevention.

Stroke is a leading cause of mortality and disability, and cardiac embolism accounts for one-third of all ischaemic strokes. Thirty per cent of strokes are cryptogenic. In this setting, echocardiography is essential in the diagnosis, treatment, and prevention of embolic stroke of undetermined source since it is a widely available, safe, and inexpensive tool. Transthoracic echocardiography and transoesophageal echocardiography, furthermore, are proven to change therapeutic management leading to initiation of anti-coagulation, anti-microbial therapy, patent foramen ovale (PFO) closure, or cardiac tumour resection. The most common cardioembolic sources include left atrial appendage thrombus, left ventricular thrombus, vegetations in endocarditis, paradoxical embolization in PFO, prosthesis thrombosis, and intracardiac tumours. Although the presence of a cardioembolic source only represents a risk factor for an ischaemic stroke, it could not assure the certain or the unique cause of the event. The purpose of this review is to underline the importance of echocardiography and overview the main sources of cardiac embolism and the echocardiographic features.

Renal cell carcinoma with intracardiac tumor thrombus extension: Radical surgery yields 2 years of postoperative survival in a single-center study over a period of 30 years.

Renal cell carcinoma (RCC) with tumor thrombus extension into the right atrium (level IV) is a rare life-threatening clinical condition that can only be managed by means of a combined urological and cardiac surgical approach. The early and late outcomes of this radical treatment were analyzed in a large single-institution series over a period of 30 years. In 37 patients with RCC and intracardiac tumor thrombus extension, nephrectomy was performed followed by the extraction of the intracaval and intracardiac tumor thrombus under direct visual control during deep hypothermic circulatory arrest (DHCA). Recently, in 13 patients, selective aortic arch perfusion (SAAP) was instituted during DHCA. In all patients, precise removal of the tumor thrombus was accomplished in a bloodless field. The mean duration of isolated DHCA was 15 ± 6min, and 31.5 ± 10.2min in the case of DHCA + SAAP, at a mean hypothermia of 22.7 ± 4°C. In-hospital mortality was 7.9% (3 patients). In Kaplan-Meier analysis, the estimated median survival was 26.4 months whereas the 5-year cancer-related survival rate was 51%. Despite its complexity, this extensive procedure can be performed safely with a generally uneventful postoperative course. The use of cardiopulmonary bypass with DHCA, with the advantage of SAAP, allows for a safe, precise, and complete extirpation of intracaval and intracardiac tumor mass. Late outcomes after radical surgical treatment in patients with RCC and tumor thrombus reaching up in the right atrium in our series justify this extensive procedure.

BRUGADA-LIKE ECG PATTERN AND TUMORS INVOLVING RIGHT VENTRICULAR OUTFLOW TRACT - CASE SERIES AND LITERATURE REVIEW.

Mechanical heart compression, including that from mediastinal tumor, may cause Brugada-like ECG pattern. Such ECG pattern might also be observed in intracardiac tumor obstructing the right ventricular out"ow tract (RVOT). Eight cases with Brugada-like ECG and tumors involving RVOT have been described so far; 4 growing in the mediastinum (with one example of in"ammatory mass), 3 with intracardiac location, and 1 being an organized pericardial hematoma. The authors present other 3 cases of intracardiac metastatic tumors in RVOT and Brugada-like ECG pattern with coved ST-segment elevation in the right precordial leads. All patients had negative history of cardiovascular disease or familiar malignant arrhythmia occurrence. ECG were done routinely; none of the patients had chest pain or an increased level of cardiac troponins. In all patients, neoplastic disease was at advanced stage. A 76-year-old male, had a history of four neoplasms: bladder cancer was being treated with chemotherapy, while prostate, tongue, and lung cancers had been resected years ago and no signs of local relapse were found. A 78-year-old female, was diagnosed with colon cancer 1 month after an episode of venous thromboembolism. Six months after the resection of cancer, second focus of adenocarcinoma was found in the rectum. Third patient, a 65-year-old-male had undergone nephrectomy for renal cancer a year before cardiac metastasis diagnosis.