Primary Cardiac Tumors Research Articles

Clinical characteristics, diagnostic methods and results of surgically treated histologically benign cardiac myxomas.

Myxomas are primary cardiac tumours that may be detected incidentally due to embolic events, intracardiac obstructive features or non-specific structural symptoms. The aim of this study was to share our experience of clinical features, diagnostic methods, surgical procedures and postoperative follow up of surgically treated cardiac myxomas. Data of 34 patients who underwent surgery for a cardiac myxoma between January 2006 and June 2022 were retrospectively analysed. Group 1 (n = 19) consisted of patients who were symptomatic and group 2 ( n = 15) patients were asymptomatic. The medical records of the patients, their clinical status, diagnostic methods, operation information and postoperative course data were collected and recorded. A total of 34 patients (16 female; mean age 54.5 ± 8.8 years) underwent cardiac myxoma resection with cardiopulmonary bypass. Fifteen (44.1%) patients were diagnosed incidentally with asymptomatic myxoma. An additional cardiac surgical procedure was performed in six patients (17.7%). The 34 cardiac myxomas that were surgically resected were localised in the left atrium in 25 patients (73.5%) and in the right atrium in nine patients (26.5%). Patients' most common symptoms were dyspnoea (42.1%), palpitations (21.1%), ischaemic stroke (15.8%) and syncope (10.5%). There was no incident of 30-day mortality and no recurrence was observed in any patient during the follow-up period. The duration of surgical intervention in symptomatic patients was significantly shorter than in asymptomatic patients (p = 0.0001), but there was no statistical difference in terms of characteristics. Myxomas are benign tumours, but they are serious pathologies that require early treatment because of signs of obstruction, embolic complications and confusion, with left atrial thrombus in the differential diagnosis.

A Rare Case of Simultaneous Cardiac Papillary Fibroelastoma and Cardiac Myxoma

A 55-year-old female was seen for a cardiac workup due to anxiety and a cardiac tumor was subsequently found on an echocardiogram. The tumor measured 3x2cm in the left atrium and was thought to be cardiac myxoma. Gross and histological findings revealed simultaneous cardiac myxoma and papillary fibroelastoma. The latter caused a decreased ejection fraction with excision requiring a mitral valve replacement. Primary cardiac tumors are rare, and most are benign with over 50% being cardiac myxomas. We experienced an exceptionally rare case of two separate simultaneous cardiac tumors in a patient.

Thirty-five years of single-center experience in cardiac myxoma surgery and related postoperative complications.

Myxoma is the most common benign primary cardiac tumor. To present a single center's 35-year experience with myxoma surgery in terms of surgical technique and postoperative complications. The data of 166 patients (56.7 ±12.6 years old, 68.1% female) with surgically removed myxoma were retrospectively analyzed. Information on blood transfusions, additional procedures and postoperative complications was collected. A median sternotomy was performed in 97.5% of patients. A right mini-thoracotomy was performed in 4 patients. Most (95.2%) patients were semi-urgent. The reason for truly urgent surgery was hemodynamic instability in 4.8% of patients. Crystalloid cardioplegia was used in 62% of cases. Postoperative complications were observed in 12% of patients. One death was observed on the first postoperative day. During hospitalization, 6 deaths (3.6%) were observed. The most common cause of death was multisystemic organ failure. There were no gender differences in mortality and complications. Surgical treatment of myxomas is a relatively safe procedure with a mortality rate comparable to other types of cardiac surgery. The postoperative complication rate is low. Gender has no influence on the complication rate or type.

A case study on right ventricular-myxoma: An unusual presentation

Myxomas originating from the right ventricle are even less common in pediatric patients. It's important to note that right ventricular myxomas are rare, and the symptoms and complications can vary from person to person. Prompt diagnosis and treatment are crucial to prevent potentially life-threatening complications. Treatment typically involves surgical removal of the tumor, and in some cases, additional measures may be necessary to address any associated complications or damage caused by the tumor. Objective: This case study aims to present a rare and noteworthy case of right ventricle myxoma in a 15-year-old boy, emphasizing the challenges in diagnosis, treatment, and the successful outcome achieved. Methods: Detailed clinical, imaging, and surgical data were collected and analyzed for a 15-year-old male patient presenting with atypical cardiac symptoms. Diagnostic procedures included 2D ECHO, X-RAY, HRCT-Thorax along with pulmonary Angio leading to the identification of a right ventricle myxoma. Incidences: Primary tumors of the heart are rare with an incidence of 0.02–0.05%. Cardiac MYXOMAS are rare BENIGN TUMORS that account for nearly 50% of all adult primary Cardiac tumors. Approximately 75–80% of myxomas are in the LEFT ATRIUM,10–20% are in the RIGHT ATRIUM, and 5–10% are in both ATRIA or either VENTRICLE. Myxomas arising from the RIGHT-VENTRICLE are extremely rare and characterized by the presence of a gelatinous mass in the right ventricle. The exact incidence of right ventricular myxoma is not well established, as it is a rare condition and limited data is available. The condition is often diagnosed incidentally during investigations for other cardiac conditions or when patients present with symptoms such as chest pain, shortness of breath, palpitations, or heart murmurs. Results: The patient, initially presenting with palpitation, underwent successful surgical excision of the right ventricle myxoma. Postoperative recovery was uneventful, and the patient demonstrated significant improvement in symptoms. Conclusion: Right ventricle myxomas are exceptionally rare in pediatric patients, posing unique diagnostic challenges. This case underscores the importance of considering cardiac tumors even in younger populations when presented with unexplained cardiac symptoms. Timely diagnosis and surgical intervention contributed to a favorable outcome, highlighting the successful management of right ventricle myxoma in adolescents.

Dispne ile başvuran bir kronik obstruktif akciğer hastasında intrakardiyak kitleden tanı alan skuamoz hücreli karsinom olgusu

Primary cardiac tumor is a rare disease with an incidence ranging from 0.001% to 0.03%. Cardiac metastases are more common than primary tumors. The most frequently metastasized tumors are lung, breast, kidney cancers and melanomas. Several published case reports have documented non-small cell lung cancer (NSCLC) patients with neoplastic heart invasion. However, the number of small cell lung cancer (SCLC) cases with neoplastic cardiac invasion is limited. In this article, we present our case of non-small cell lung cancer with cardiac invasion who presented to the emergency department with dyspnea.

A Case of Hip Chondrosarcoma Metastasis to the Right Ventricle

Background. While primary cardiac tumors occur rarely, it is observed that cardiac metastases are almost 20 times more common. These are, however, observed during autopsy and seldom missed in living patients. Patients with cardiac metastases present commonly with dyspnea on exertion or pleuritic chest pain. Most common site of intracardiac metastases is right atrium.
 Case presentation. Here we present a case of a 21-year-old male who presented for a routine check-up to the cardiac outpatient department for assessment of fitness for chemotherapy. He was a known case of right hip chondrosarcoma for 2 years, being treated with Ayurvedic medications. He had no symptoms related to the respiratory or cardiac system. He was afebrile during the examination, however, was restricted to a bed due to the pain in his right hip. On evaluation, the patient was diagnosed with a right ventricular thrombus extending to the pulmonary artery (PA) causing an impending pulmonary embolism. On opening the right atrium and PA, we found a loose, whitish-grey mass, adherent to the right ventricular papillary muscle, entangling the chordae. The tricuspid valve leaflets were also found to be tethered to this mass. The mass was found to extend to the PA beyond the leaflets, however, the leaflets were free of the tissue. The mass was extracted piece-meal through right atrium and PA approach. Histopathology revealed metastatic chondrosarcoma tissue.
 Conclusion. Cardiac metastases of chondrosarcoma are a rare condition, and can be often asymptomatic. Such patients often present with dyspnea, and imaging may not be able to rightly identify the cause. A high index of suspicion is necessary in patients with a known current or prior malignancy before committing to surgical intervention.

Transcatheter Biopsy of Primary Cardiac Tumors Guided by Echocardiographic and Fluoroscopic Imaging.

Transcatheter Biopsy of Primary Cardiac Tumors Guided by Echocardiographic and Fluoroscopic Imaging.

Huge right atrial myxoma manifesting with dyspnea and right-sided heart failure: A case report

Although cardiac myxomas are the most prevalent type of primary cardiac tumors, barely they originated from the right atrium and generally are asymptomatic in such cases. Here we described a case of a 24-year-old male with right atrial myxoma.

A monster into the heart: an unusual presentation of cardiac leiomyosarcoma

BackgroundMalignant primary cardiac tumors are infrequent and can lead to an unfavorable prognosis if not identified and treated promptly. Early detection and prompt treatment of malignant primary cardiac tumors are crucial for a better prognosis. This article presents a case of primary cardiac leiomyosarcoma and reviews the literature on this topic.Case presentationFemale patient that developed recurrent pericardial effusion and hemodynamic instability caused by a cardiac tumor, later identified as leiomyosarcoma. Multidisciplinary treatment was administered to the patient.ConclusionsThe initial approach to this type of pathology should include multimodality imaging to establish a prompt diagnosis leading to complete standard treatment, to minimize risks to the patient's heart function which may include resection with complete margins of the neoplasm, otherwise the prognosis may be poor.

The benign nature and rare occurrence of cardiac myxoma as a possible consequence of the limited cardiac proliferative/ regenerative potential: a systematic review

BackgroundCardiac Myxoma is a primary tumor of heart. Its origins, rarity of the occurrence of primary cardiac tumors and how it may be related to limited cardiac regenerative potential, are not yet entirely known. This study investigates the key cardiac genes/ transcription factors (TFs) and signaling pathways to understand these important questions.MethodsDatabases including PubMed, MEDLINE, and Google Scholar were searched for published articles without any date restrictions, involving cardiac myxoma, cardiac genes/TFs/signaling pathways and their roles in cardiogenesis, proliferation, differentiation, key interactions and tumorigenesis, with focus on cardiomyocytes.ResultsThe cardiac genetic landscape is governed by a very tight control between proliferation and differentiation-related genes/TFs/pathways. Cardiac myxoma originates possibly as a consequence of dysregulations in the gene expression of differentiation regulators including Tbx5, GATA4, HAND1/2, MYOCD, HOPX, BMPs. Such dysregulations switch the expression of cardiomyocytes into progenitor-like state in cardiac myxoma development by dysregulating Isl1, Baf60 complex, Wnt, FGF, Notch, Mef2c and others.The Nkx2–5 and MSX2 contribute predominantly to both proliferation and differentiation of Cardiac Progenitor Cells (CPCs), may possibly serve roles based on the microenvironment and the direction of cell circuitry in cardiac tumorigenesis. The Nkx2–5 in cardiac myxoma may serve to limit progression of tumorigenesis as it has massive control over the proliferation of CPCs. The cardiac cell type-specific genetic programming plays governing role in controlling the tumorigenesis and regenerative potential.ConclusionThe cardiomyocytes have very limited proliferative and regenerative potential. They survive for long periods of time and tightly maintain the gene expression of differentiation genes such as Tbx5, GATA4 that interact with tumor suppressors (TS) and exert TS like effect. The total effect such gene expression exerts is responsible for the rare occurrence and benign nature of primary cardiac tumors. This prevents the progression of tumorigenesis. But this also limits the regenerative and proliferative potential of cardiomyocytes. Cardiac Myxoma develops as a consequence of dysregulations in these key genes which revert the cells towards progenitor-like state, hallmark of CM. The CM development in carney complex also signifies the role of TS in cardiac cells.

Combination of Takotsubo cardiomyopathy with cardiac myxoma: a case report

Introduction. In the Russian literature, we have not found data on the combination of takotsubo syndrome (TTS) and left atrial (LA) myxoma. The work analyzed our own clinical case. TTS is diagnosed in 1,0-2,5% of patients hospitalized with suspected acute coronary syndrome (ACS). Primary cardiac tumors occur in cardiology with a frequency of up to 0,2%. The most common of them are LA myxoma.Brief description. The patient was admitted to the clinic with suspected ACS. During the diagnostic search, both ACS and acute myocarditis were ruled out. An incidental finding during an echocardiographic study was LA myxoma.Discussion. Due to a stressful situation, the patient developed symptoms similar to myocardial infarction. Based on coronary angiography data (normal coronary arteries, rapid restoration of myocardial contractility, no characteristic myocardial changes, myocardial infarction and acute myocarditis were excluded and TTS was established. LA myxoma was an unexpected finding.Myxoma may contribute to TTS, presumably through central mechanisms regulating the activity of the cardiovascular system. Also important is the systemic inflammation caused by myxoma, which reduces the threshold for the development of Takotsubo cardiomyopathy in response to stress factors.

Cardiac Metastases of Recurrent Infiltrating Amelanotic Fusocellular Malignant Melanoma: Case Report

Melanoma is one of the most aggressive types of skin cancer. The malignant amelanotic fusocellular melanoma subtype is an uncommon presentation, however, it has a high rate of cardiac involvement, it presents asymptomatic or with symptoms ranging from nonspecific to cardiac tamponade or heart failure. We present a case of a 48-year-old man with recurrent infiltrating amelanotic fusocellular malignant melanoma, with incidentally visualized biventricular lesion and right atrium, with cardiac tamponade with subsequent fluoroscopy-guided pericardiocentesis. Primary cardiac tumors have a prevalence of 1:2000 autopsies and secondary cardiac tumors of 1:100 autopsies, remaining as a secondary: primary ratio of 20:1. In patients with this type of melanoma, although cardiac metastases are common, the diagnosis of them is rare, when it occurs, the disease is very advanced and the treatment is usually palliative.

Cardiac Papillary Fibroelastoma, an Unusual Localization in an Asymptomatic Patient with Lieberkühn Type Adenocarcinoma of the Rectum: A Case Report

Cardiac papillary fibroelastoma is histologically a benign endocardial neoplasm. Though CPF has long been reported as the second most common primary cardiac neoplasm, it has since been pulled ahead of cardiac myxomas, largely due to evolving cardiac imaging modalities. We present a case of CPF originating from an unusual localization; the junction of the posterior mitral valve and endocardium of the left ventricle measuring 15 x 20mm, which was detected by a routine pre-evaluation transthoracic echocardiography before chemotherapy and treated by surgical mass excision in an asymptomatic female patient diagnosed with a Lieberkühn type adenocarcinoma of the rectum with a locoregional extension.

Need an endomyocardial biopsy? Call your electrophysiology team

Abstract Introduction With significant advances in targeted chemotherapeutic options for soft tissue tumours, obtaining a tissue diagnosis is more important than ever. Primary and secondary cardiac tumours are rare [1] and endomyocardial biopsy of these tumours has been sparsely reported [2-9]. Advanced tools in cardiac electrophysiology happen to lend themselves well to cardiac tumour biopsy. Our electrophysiology (EP) guided endomyocardial biopsy (EMB) service includes electrophysiologists, cardio-oncology specialists, cardiac imaging specialists, oncologists, and pathologists. For the last 9 years all targeted endomyocardial biopsies in our tertiary institution have been undertaken by our EP guided EMB service with extensive experience in electroanatomical mapping (EAM), visualisable sheaths and integrated intracardiac echography (ICE). We report the findings. Methods We performed a retrospective review of all cases referred to the EP guided endomyocardial biopsy service at our centre and identified those with attempts to biopsy a cardiac mass. We describe the incorporation of advanced electrophysiology techniques utilised by the service. Specifically, the use of intracardiac echocardiography integrated into an electro-anatomical mapping system and bioptome delivery via a visualisable, deflectable sheath. This allows for the real time 3D visualisation of the target mass, surrounding anatomy and position of the bioptome during biopsy. Results Of 38 patients who underwent targeted biopsy at our centre, a total of 8 patients (5 female; mean ± SD age 54 ± 21 years) were referred for cardiac tumour biopsy, undergoing a total of 9 procedures (mean ± SD duration 116 ± 21 mins). Table 1 details the patient’s pertinent history and underlying pathology, the tumour site, possible alternative biopsy sites, a summary of techniques and technologies utilised and the eventual diagnosis. In 100% of cases, tissue was sampled from the desired location, though in two patients the histological result was inconclusive due the retrieval of necrotic tissue or fibrotic tissue only. This translated into a definitive histopathological diagnosis in 75% of patients. One patient experienced a transient right bundle branch block during the procedure, in all others the procedures were uncomplicated. Image 1 exemplifies some of the cases with pre-procedure imaging results and intra-procedural views from both ICE and the EAM. Conclusion In this study, our approach to utilising the combination of intracardiac echocardiography integrated into an electro-anatomical mapping system and bioptome delivery via a visualisable, deflectable sheath appears safe, and provides unparalleled accuracy in endomyocardial sampling of cardiac tumours.Table 1Image 1

Abstract 17857: Demographic Disparities and Survival Outcomes in Primary Cardiac Tumors

Introduction: Secondary cardiac tumors are not uncommon as they are seen in almost 14% of patients with multiple distant metastases, the most common primaries being lung, breast, and hematological malignancies. Whereas, primary cardiac tumors are rare, and only about 10-25% of these are primary malignant cardiac tumors (PMCT). With this study, we aim to analyze the demographic disparities and the determinants of survival in this infrequent group of cardiac neoplasms. Methods: We obtained data from the Surveillance, Epidemiology, and End Results (SEER) database, 17 registries, Nov 2022 sub (2000-2020) for PMCT. A total of 713 samples were obtained after excluding patients with unknown age, race, and survival. Cox proportional hazard regression was used for multivariate survival analysis. Results: The mean age at diagnosis of PMCT was 55.13 ± 20.47 years, with the standard deviation reflecting a wide range and dispersion. Males accounted for 51.75% of the patients. Racial demographics showed a predominance of White (61%), followed by Hispanic (18.65%), Asian/pacific islander (10.09%), and Black (9.67%) populations. The most predominant types were sarcomas (62.27%) and lymphomas (27.48 %), with the rest 10.23% including tumors like mesothelioma and adenocarcinoma among others. Kaplan-Meier method was used to estimate overall survival (OS) at 1, 3 and 5 years to be 50.8%, 26.6% and 18% respectively. Cox regression demonstrated statistically significant differences in survival only with age and histopathology. Older patients [HR 1.008 (1.004-1.012)] had worse OS. Compared to sarcomas, lymphomas [HR 0.45 (0.36-0.55)] had better OS, while the group comprising the other 10.23% of PMCTs [HR 1.52 (1.17-1.96)] had worse OS. No statistically significant differences in survival were noted between males and females, and also between different racial groups. Conclusions: Despite being rare malignancies, PMCTs continue to have a poor prognosis. Though sex and ethnicity do not influence survival, older patients with sarcomas and other rare PMCTs have poorer outcomes. Further investigations aimed at improving survival in these at-risk groups are warranted. This information will also aid providers in advanced care planning.

Abstract 16580: Cardiovascular Disease in Cushing Syndrome, Conns Syndrome and Malignant Pheochromocytoma: Which Adrenal Disorder is Associated With Thromboembolism and Cardiac Tumors?

Background: Adrenal disorders result in worse cardiovascular outcomes. Literature suggests that Cushing (CUSH) and Conn’s syndrome (CONS) are associated with coronary artery disease, congestive heart failure (CHF), arrhythmia and stroke. But the date comparing the association of various adrenal disorders with cardiovascular disease (CVD) is limited. We reviewed the prevalence of CVD in CUSH, CONS, and malignant pheochromocytoma (MPH) and explored their prognosis. Methods: A retrospective cohort analysis was conducted using National Inpatient Sample 2016-2020. Cohorts of patients hospitalized for CUSH, CONS, MPH and CVD were identified using respective ICD-10 codes. Primary endpoint was outcomes of CUSH, CONS, and MPH in terms of CVD. Multivariate logistic regression model was used to assess prognostic outcomes. Results: 58060 patients were admitted with CUSH, 3420 with CONS, and 1445 with MPH. CONS has the highest odds of association with CVD (OR 4.87, CI 3.74-6.3, p<0.001), followed by CUSH (OR 3.45, CI 3.3-3.6, <0.001), and MPH (OR 3, CI 2.1-4.3, p<0.001). Upon subgroup analysis of CVD, we observed that CUSH has strongest association with venous thromboembolism (VTE, OR 2.56, CI 2.3-2.8, p<0.001), pulmonary embolism (PE 2.3, OR 2.03-2.6, p<0.001), and CHF (OR 2.2, CI 2.1-2.3, p<0.001). CONS has highest odds of association with primary cardiac tumors (9.2, CI 1.29-65.3, p=0.027), hypertension (HTN, OR 2.8, CI 2.34-3.3, p<0.001), and atrial fibrillation (AF, OR 1.66, CI 1.32-2.06, p<0.001). While MPH is strongly associated with higher odds of pulmonary edema (OR 3.89, CI 1.71-8.8, p<0.01), and HTN (OR 2.8, CI 2.1-3.6, p<0.001). Conclusion: Most prevalent CVD in adrenal disorders are HTN, IHD, CHF, and AF. CONS has highest prevalence and strongest association with CVD followed by CUSH and MPH. CUSH is strongly associated with thromboembolism while CONS with primary cardiac tumors, and MPH with pulmonary edema.

Abstract 14125: Tricuspid Mass- Solving a Curious Case of Li-Fraumeni Syndrome

Background: Primary cardiac tumors are extremely rare (0.001-0.03%), but can have significant embolic, obstructive, and arrhythmic consequences. Li-Fraumeni syndrome (LFS) is an autosomal dominant genetic syndrome characterized by multiple malignancies, including osteosarcomas, CNS tumors, and adrenocortical, breast, and soft tissue sarcomas. We report diagnostic challenges of a patient with Li-Fraumeni syndrome found to have tricuspid valve mass and atrial septal defects. Case: 30-year-old female with Li-Fraumeni syndrome (ovarian cancer at 6 years,osteosarcoma and soft tissue sarcoma at 16 years, bilateral breast cancer at 26 years) found to have a tricuspid valve leaflet mass, as well as two atrial septal defects on surveillance echocardiography. There was concern for a primary cardiac tumor such as sarcoma or rhabdomyoma. She underwent surgical resection with biopsy showing organized mural thrombus. Decision making: Li-Fraumeni syndrome is a rare autosomal dominant cancer-predisposing syndrome, which can present with various tumor types. Cardiac tumors are extremely rare and have been limited to case reports. Although multimodality imaging is now more readily available, some cases remain diagnostic challenges if imaging is non-conclusive, histopathological studies remain the gold standard. In our case, the mass demonstrated mild enhancement in the post-gadolinium images suggestive for primary cardiac tumor versus a secondary metastatic cancer (Figure). A histopathological analysis by surgical resection demonstrated an organized thrombus. Conclusion: Diagnosis of cardiac masses in the setting of hereditary cancer syndromes such as LFS are rare and remain a challenge given the increased likelihood of primary or metastatic lesions. When imaging is inconclusive, further invasive investigations are warranted since it can alter the clinical management.

Abstract 17111: Spontaneous Regression of Cardiac Angiosarcoma in a 17-Year-Old Male

Description of Case: A fit and healthy 17-year-old male was admitted with a 10-day history of chest pain and SOB. He was taking no medication and had no significant past medical or family history. Clinical examination revealed signs of pericarditis with widespread ST elevation and PR depression on ECG. TTE showed a moderate, global pericardial effusion. He was treated for pericarditis with anti-inflammatory medication and discharged with follow up a few weeks later. Repeat TTE demonstrated a mass in the pericardial space adjacent to the RV free wall. Urgent CMR was requested to exclude malignancy and showed a large, irregular heterogenous mass adjacent to the RA and RV with invasion into the RV free wall. CMR characterisation including first pass perfusion was indeterminate. CT TAP showed no distant metastases. Percutaneous biopsy was non diagnostic and serial CMR 3 weeks later showed significant reduction of the mass. PET-CT confirmed reduction in the size of the mass although there was moderate heterogeneous FDG uptake, more suggestive of malignancy rather than a benign lesion. Given the apparent resorption of the mass, repeat CMR was planned 3 months later. Repeat CMR (6 months after initial CMR) showed significant increase in the size of the cardiac mass. Surgical biopsy confirmed cardiac angiosarcoma and was unresectable. After MDT discussion, the patient was started on doxorubicin, dexrazoxane and pazopanib. This has failed to control the tumour, and he has now commenced a trial of paclitaxel. Discussion: Spontaneous regression of cardiac rhabdomyoma in infants has been described, but this does not extend to other primary cardiac tumours or adults. The re-emergence and significant increase in size of the mass suggests initial regression may have been a percutaneous biopsy effect causing collapse or resorption of the necrotic core. Suggested mechanisms include disruption of the tumour feeding artery during biopsy or activation of the immune system.

Abstract 16992: Superior Vena Cava Syndrome in a Patient With Metastatic Primary Cardiac Diffuse Large B-cell Lymphoma - Case Report

Introduction: Primary cardiac tumors are uncommon, with an estimated incidence of 0.02%. Primary cardiac lymphomas (PCL), a subtype of non-Hodgkin lymphoma, account for about 10% of malignant and 1% of all primary cardiac tumors. Diffuse large B-cell lymphomas (DLBCLs) emerge as the predominant histological subtype within PCL cases. Case presentation: 54-year-old White male with no medical history presented with a few weeks of exertional dyspnea, nonproductive cough, and neck fullness. Exam was notable for tachycardia, jugular venous distension, bilateral upper extremity edema, and distended chest wall vasculature. Labs showed elevation of alkaline phosphatase and CRP. CT of the chest showed a right atrial mass extending into and obstructing the superior vena cava (SVC) and mediastinal lymphadenopathy (Panel A & B). Coronary CTA performed a few years earlier showed normal interatrial septum (Panel C) and coronary arteries. Transthoracic echo (TTE) confirmed a 7.6 x 5.0cm mass visualized in the interatrial septum extending to both atria. Cardiac MRI redemonstrated a large cardiac mass with malignant features suggestive of cardiac lymphoma vs angiosarcoma (Panel D-F). PET-CT showed enhanced fluorodeoxyglucose (FDG) uptake within cardiac mass, mediastinal, and intraabdominal lymph nodes (Panel G). Lymph node biopsy confirmed DLBCL. Patient underwent three cycles of EPOCH (etoposide phosphate, prednisone, vincristine sulfate, cyclophosphamide, doxorubicin hydrochloride) chemotherapy with excellent response. TTE following cycle 2 demonstrated decreased tumor size, and PET-CT after cycle 3 confirmed complete resolution of lymphadenopathy and minimal residual intracardiac FDG uptake. Conclusion: SVC syndrome can be a rare presentation of PCL. Multimodality imaging is important in cardiac mass characterization, diagnosis, treatment planning, and follow-up. Timely diagnosis and initiation of chemotherapy results in improved outcomes.

Abstract 18126: A Rare Case of a Left Ventricular Papillary Fibroelastoma With an Initial Presentation of Unilateral Vision Loss

A 59-year-old female with hypertension presented with acute onset of decreased vision in the right eye. She reported waking up 3 days prior to admission with sudden onset of shadow in the right eye appearing like a falling curtain which eventually progressed to total visual loss. She also complained of headaches and palpitations. The patient denied pain, nausea, vomiting, photophobia, changes in speech, aphasia, facial weakness, arm or leg weakness or numbness. She states that this is her first experience of vision loss. At the time of admission, she was hypertensive to 170/100s and started on amlodipine 5mg and losartan 25mg. Neurology and ophthalmology were consulted for concerns of stroke and new vision loss. She was started on heparin drip, aspirin 81mg, atorvastatin 80mg, and methylprednisolone 1g. Ocular examination revealed right brachial retinal artery occlusion. MRI brain showed chronic microvascular changes, but no acute intracranial hemorrhage or infarction. CT angiogram of the neck showed mild soft plaque in right carotid bifurcation. Transthoracic echocardiogram revealed a 1.6cm x 1.6cm left ventricular mobile mass which appeared to be attached to the posteromedial papillary muscle and left ventricle ejection fraction of 60%. The patient underwent open heart surgery for removal of the mass. The mass was confirmed to be a papillary fibroelastoma attached to the left ventricular trabeculum. The mitral valve and subvalvular apparatus were not involved. New onset of stroke or acute vision loss is generally a result of thromboembolism. Most times it can be caused by a clot formed in a patient with atrial fibrillation. In other cases, it can be due to a ruptured cholesterol plaque. However, primary cardiac tumors are rare, especially ones on the papillary muscle. We presented a rare case of papillary fibroelastoma that possibly embolized causing acute unilateral vison loss. This case reinforces the importance of cardiac imaging in new stroke or acute vision loss.