Primary Malignant Cardiac Tumors Research Articles

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.

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.

Reduced Risk of All-Cause, Cancer-, and Cardiovascular Disease-Related Mortality among Patients with Primary Malignant Cardiac Tumors Receiving Chemotherapy in the United States.

Primary malignant cardiac tumors (PMCTs) are rare but lethal neoplasms. There are limited evidence-based treatment guidelines for PMCTs. We evaluated the relation of chemotherapy with mortality outcomes in patients with PMCTs in the United States. Data were from patients aged ≥ 20 years from the Surveillance, Epidemiology, and End Results program who were diagnosed with PMCTs from 2000 to 2020. Cox regression, competing risk, and propensity score analyses were performed to estimate hazard ratios (HR) and confidence intervals (CI). About 53% of the 563 patients with PMCTs received chemotherapy as the first course of treatment. During a mean follow-up of 24.7 months (median: 10), 458 deaths occurred with 81.7% and 9.4% due to cancer and cardiovascular disease (CVD), respectively. In models adjusted for sociodemographic and clinico-pathophysiological factors including histology, receipt of chemotherapy was associated with low risk for all-cause (HR: 0.56, 95%CI: 0.45-0.69), cancer (HR: 0.63, 95%CI: 0.50-0.80) and CVD mortality (HR: 0.27, 95%CI: 0.12-0.58). Patients who had both chemotherapy and surgery had the lowest risk for all-cause and cancer mortality. This study suggests that the subpopulations of patients with PMCTs who receive chemotherapy may have better prognosis than those who do not receive this therapy regardless of histology.

Survival and analysis of prognostic factors for primary malignant cardiac tumors based on the SEER database.

The purpose of this study was to use the Surveillance, Epidemiology, and End Results (SEER) database to evaluate the survival rate of primary malignant cardiac tumors (PMCTs), assess the risk factors affecting survival, and calculate the number of PMCT cases in recent years. SEER 22 registries were used to calculate the number of cases PMCT. Data on age, sex, race, marital status, tumor size, the American Joint Committee on Cancer (AJCC) stage, lymph node involvement, metastasis, treatment, and survival were collected to analyze the survival and prognostic factors of SEER 17 registries. Using the Kaplan-Meier estimation method, a survival curve was obtained according to the influencing factors, and a multivariable Cox regression model was established. In recent years, the average annual number of PMCT cases was 20.56 ± 7.12, significantly higher than the average before 2004 (P = 0.015; 95% CI 1.14-8.98). The 1-, 3-, and 5-year survival rates were 45.6%, 18.8%, and 11.2%, respectively. Multivariate analysis revealed that age (risk ratio [HR], 2.047; 95% CI 1.381-3.034), AJCC stage III (HR, 1.786; 95% CI 1.123-2.839), AJCC staging with distant metastasis (HR, 2.666; 95% CI 1.509-4.709), no chemotherapy (HR, 2.011; 95% CI 1.561-2.590), and tumor size larger than 99mm (HR, 1.766; 95% CI 1.132-2.756) were independent risk factors for poor prognosis. Only age over 76years and distant metastasis were independent risk factors for prognosis in the chemotherapy group. In recent years, the annual number of patients with PMCT has increased significantly. Due to developments in chemotherapy, we should re-evaluate the traditional tumor staging and prognostic risk indicators to improve clinical applications.

Age and treatment disparities in survival of primary malignant cardiac tumors: an analysis of over 40 years and 500 patients.

Primary malignant cardiac tumors (PMCTs) are rare and tend to have a poor prognosis, due to their aggressive biological behavior and the inadequate expertise with the disease. This article compares the survival of patients with PMCT subtypes in the United States across age and treatment groups. Data of 529 patients diagnosed with PMCTs were analyzed. Chi-squared test was used to assess significance of the differences between proportions in demographic and tumor characteristics by age and treatment. Cox regression analysis was used to estimate survival from the Surveillance, Epidemiology, and End Results (SEER) follow-up data. Survival rates for PMCTs differed significantly between age groups, with patients younger than 20 years surviving significantly longer than those older than 80 years. The median survival times of all patients with PMCTs were 22.5, 11, 5, and 1 month for ages less than 20, 20-50, 51-80, and greater than 80 years, respectively (global log-rank P=0.0026). In the treatment cohort, for all tumors [hazard ratio (HR) 1.52, P<0.001], sarcomas (HR 1.83, P=0.002), and other tumors (HR 2.24, P=0.017), survival was lower in patients who did not receive treatment than in those who received only surgery. Survival after diagnosis of sarcoma was lower in patients who received radiotherapy only than in those who received surgery only (HR 1.49, P=0.046). However, there was no significant association between treatment and survival for lymphoma and mesothelioma. This study confirms that PMCTs have limited treatment options and poor patient survival, especially for elderly patients and patients who receive no treatment. And patients with PMCTs of any age, whether treated or not, have poor survival rates. Techniques for early diagnosis and treatment may be necessary. Surgical treatment should have a higher priority for future treatment of patients with sarcomas.

P270 THE IMPORTANCE OF INTEGRATED IMAGING IN THE DIFFICULT DIFFERENTIAL DIAGNOSIS OF A LEFT INTRACARDIAC MASS IN YOUNG ADULT

Abstract The patient is a 34–year–old man who comes to the emergency room with mild hemoptysis, asthenia, low–grade fever, night sweats and weight loss. Cardiac auscultation reveals a diastolic murmur at the cardiac apex. Transthoracic and transesophageal echocardiograms are performed and show an isoechoic thickening encompassing the posterior leaflet, cranially involving the atrial septum and the lateral wall up to the inlet of the left atrial appendage and inferiorly involveing the subvalvular apparatus with diffuse thickening and chordal fusion. Functionally it determines a moderate mitral stenosis with an average gradient of 10 mmHg and a valve area of ​​1.1 cm2 and a mild–moderate regurgitation with an anteriorly directed eccentric jet. At this point we needed to make the differential diagnosis considering as possible etiologies infective endocardic vegetation, thrombosis, primary and secondary neoplasms, aseptic vegetation and inflammatory or rheumatic valvulitis. We therefore begin our diagnostic work–up by performing a scintigraphy with labeled leukocytes, which does not show the presence of hyperuptake areas referable to infective endocarditis, and a PET scan which shows, in addition to the increased uptake at the cardiac level, an area of ​​greater concentration of the metabolic tracer in the gastric area (between the body and the fundus). Given the confirmation of metabolically active tissue, the patient performed cardiac magnetic resonance with contrast medium which confirmed the presence of pathological tissue with inhomogeneous late post–contrast enhancement. After collegial discussion, due to the high risk of biopsy of the mitral lesion, which was poorly separable from the cardiac structures, given the other localization of the disease at the gastric level, it was decided to perform an EGDS with relief of an ulcerated lesion on the gastric fundus which is subjected to biopsy and histological typing. We then arrive at the diagnosis of “undifferentiated pleomorphic sarcoma”. Sarcoma is the most frequent histological type of primary malignant cardiac tumors. The clinic is characterized by constitutional symptoms, obstruction of the valve inflow or outflow tract, thromboembolism, and arrhythmias. It has a poor prognosis: 6–12 months from diagnosis. In conclusion, we reiterate the importance of integrated imaging to arrive at the final diagnosis, each method with a specific objective.

Multiparametric mapping by cardiovascular magnetic resonance imaging in cardiac tumors

BackgroundThere is a paucity of quantitative measurements of cardiac tumors and myocardium using parametric mapping techniques. This study aims to explore quantitative characteristics and diagnostic performance of native T1, T2, and extracellular volume (ECV) values of cardiac tumors and left ventricular (LV) myocardium. MethodsPatients with suspected cardiac tumors who underwent cardiovascular magnetic resonance (CMR) between November 2013 and March 2021 were prospectively enrolled. The diagnoses of primary benign or malignant tumors were based on pathologic findings if available, comprehensive medical history evaluations, imaging, and long-term follow-up data. Patients with pseudo-tumors, cardiac metastasis, primary cardiac diseases, and prior radiotherapy or chemotherapy were excluded. Multiparametric mapping values were measured on both cardiac tumors and the LV myocardium. Statistical analyses were performed using independent-samples t-test, receiver operating characteristic, and Bland–Altman analyses. ResultsA total of 80 patients diagnosed with benign (n = 54), or primary malignant cardiac tumors (n = 26), and 50 age and sex-matched healthy volunteers were included. Intergroup differences in the T1 and T2 values of cardiac tumors were not significant, however, patients with primary malignant cardiac tumors showed significantly higher mean myocardial T1 values (1360 ± 61.4 ms) compared with patients with benign tumors (1259.7 ± 46.2 ms), and normal controls (1206 ± 44.0 ms, all P < 0.05) at 3 T. Patients with primary malignant cardiac tumors also showed significantly higher mean ECV (34.6 ± 5.2%) compared with patients with benign (30.0 ± 2.5%) tumors, and normal controls (27.3 ± 3.0%, all P < 0.05). For the differentiation between primary malignant and benign cardiac tumors, the mean myocardial native T1 value showed the highest efficacy (AUC: 0.919, cutoff value: 1300 ms) compared with mean ECV (AUC: 0.817) and T2 (AUC: 0.619) values. ConclusionNative T1 and T2 of cardiac tumors showed high heterogeneity, while myocardial native T1 values in primary malignant cardiac tumors were elevated compared to patients with benign cardiac tumors, which may serve as a new imaging marker for primary malignant cardiac tumors.

Malignant Primary and Metastatic Cardiac Tumors: A Single-Center 27-Year Case Review

Background: Malignant primary cardiac tumors are exceedingly rare, and despite surgical exeresis or chemotherapy, their prognosis remains poor. Cardiac invasion by metastatic tumors, while more common, also entails an unsatisfactory outcome. This study aimed to review patients diagnosed with malignant primary and secondary cardiac tumors in a tertiary center between 1995 and 2022. Methods: Clinical data, echocardiographic, computed tomography, and magnetic resonance assessments of tumor location and morphology, histology, treatment, and survival were retrospectively analyzed. Results: Sixty malignant cardiac tumors were diagnosed: 17 primary (A) and 43 metastatic (B) tumors. A: the most common types were angiosarcoma (41%), undifferentiated sarcoma (23%), and fibrosarcoma (18%). Patients with primary tumors were younger than patients with metastatic tumors (41 ± 13 years vs. 57 ± 18 years, p = 0.001), with no significant gender difference. The most frequent presentations were heart failure (59%) and arrhythmia (23%). The most prevalent tumor location was the right heart chambers (71%), mostly in the right atrium (35%). 47% were submitted to tumor resection, and 29% received chemotherapy. The mortality rate was 82% with a median survival of 6.0 (interquartile range: 1.0–11.8) months after diagnosis (minimum of 12 days and maximum of 19 years). One patient with fibrosarcoma underwent heart transplantation and was still alive and well after 19 years. B: regarding metastatic cardiac invasion, the most common primary tumor sites were lung carcinomas (38%), thymomas (17%), and lymphomas (14%). Presentation with pericardial effusion was common (33%). The mortality rate was 72%, with a median survival of 3.6 (1.0–13.4) months (minimum of 7 days, maximum of 5 years). Conclusion: Diagnosis of metastatic cardiac tumors was more common than that of malignant primary tumors, both with a dismal prognosis. When radical exeresis is not possible, heart transplantation can be an option with a favorable outcome in carefully selected patients with sarcomas.

Metastatic patterns and prognosis of patients with primary malignant cardiac tumor.

Distant metastases are independent negative prognostic factors for patients with primary malignant cardiac tumors (PMCT). This study aims to further investigate metastatic patterns and their prognostic effects in patients with PMCT. This multicenter retrospective study included 218 patients with PMCT diagnosed between 2010 and 2017 from Surveillance, Epidemiology, and End Results (SEER) database. Logistic regression was utilized to identify metastatic risk factors. A Chi-square test was performed to assess the metastatic rate. Kaplan-Meier methods and Cox regression analysis were used to analyze the prognostic effects of metastatic patterns. Sarcoma (p = 0.002) and tumor size¿4 cm (p = 0.006) were independent risk factors of distant metastases in patients with PMCT. Single lung metastasis (about 34%) was the most common of all metastatic patterns, and lung metastases occurred more frequently (17.9%) than bone, liver, and brain. Brain metastases had worst overall survival (OS) and cancer-specific survival (CSS) among other metastases, like lung, bone, liver, and brain (OS: HR = 3.20, 95% CI: 1.02-10.00, p = 0.046; CSS: HR = 3.53, 95% CI: 1.09-11.47, p = 0.036). Patients with PMCT who had sarcoma or a tumor larger than 4 cm had a higher risk of distant metastases. Lung was the most common metastatic site, and brain metastases had worst survival among others, such as lung, bone, liver, and brain. The results of this study provide insight for early detection, diagnosis, and treatment of distant metastases associated with PMCT.

Imaging of pediatric cardiac tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Cardiac tumors in children are rare and the majority are benign. The most common cardiac tumor in children is rhabdomyoma, usually associated with tuberous sclerosis complex. Other benign cardiac masses include fibromas, myxomas, hemangiomas, and teratomas. Primary malignant cardiac tumors are exceedingly rare, with the most common pathology being soft tissue sarcomas. This paper provides consensus-based imaging recommendations for the evaluation of patients with cardiac tumors at diagnosis and follow-up, including during and after therapy.

A 20-year experience in cardiac tumors: a single center surgical experience and a review of literature.

Cardiac tumors are rare and heterogeneous entities which still remain a diagnostic and therapeutic challenge. The treatment for most cardiac tumors is prompt surgical resection. We sought to provide an overview of surgical results from a series of consecutive patients treated at our tertiary care center during almost a 20-year experience. In this single center study, 55 consecutive patients with diagnosis of cardiac tumor underwent surgical treatment from January 2002 to April 2021. Of these, 23 (42%) were male and the mean age was 62 ± 12 years. Fifteen (27%) patients were symptomatic at the time of the diagnosis, mostly for dyspnea and palpitations. The most frequent benign cardiac tumor was myxoma (32; 58%), occurring mainly in the left atrium (31; 97%). Pleomorphic sarcoma was the most frequent primary malignant cardiac tumor (4; 7%), mainly located in the ventricles (1; 25% in the left ventricle; 2; 50% in the right ventricle). In all cases of benign tumors surgery was successful with no relapses. Two (50%) pleomorphic sarcomas showed subsequent relapses. After a median follow-up of 44 months, 15 (27%) patients died. Although malignant tumors presented a limited survival, benign tumors showed a very good prognosis. Cardiac tumors require a multidisciplinary approach to guarantee a prompt diagnosis and appropriate treatment. In our surgical experience, outcome after surgery of benign tumors was excellent, while malignant tumors had poor prognosis despite radical surgery.

Racial and ethnic characteristics and cancer-specific survival in Primary Malignant Cardiac Tumors.

BackgroundThere is limited insight into the epidemiological characteristics and effect of race and ethnicity on Primary Malignant Cardiac Tumors (PMCTs).ObjectivesComparison of clinical characteristics and cancer-specific survival outcomes of major races in the United States from the Surveillance, Epidemiology and End-Result (SEER) registry.MethodsICD-O-3 codes were used to identify PMCTs for the years 1975 to 2015. Three major races were identified—“White”, “Black”, and “Asian/Pacific Islander”. Cancer-specific survival outcomes were compared using Kaplan-Meier analysis across and amongst races, based on tumor histology. A subgroup analysis of cancer-specific survival was performed between “Hispanics” and “non-Hispanics.”ResultsSeven hundred and twenty patients were identified−47% females and 79% White, mean age at diagnosis (47 ± 20 years). Black patients were significantly younger (39 ± 18 years) and presented more commonly with angiosarcomas (53%). Non-angiogenic sarcomas and lymphomas were the most common tumors in the White (38%) and Asian/Pacific Islander (34%) cohorts. For a median follow-up period of 50 (IQR3-86) months, cancer-specific survival (mean ± SD, in months) was worse in Blacks (9 ± 3) as compared to Whites (15 ± 1) and Asian/Pacific Islander (14 ± 1) (p-value; Black vs. White <0.001; Black vs. Asian/Pacific Islanders = 0.017, White vs. Asian/Pacific Islanders = 0.3). Subgroup analysis with 116 (16%) Hispanics (40% females; mean age of 40 ± 20 years) showed a longer mean cancer-specific survival of 16.9 ± 2.4 months as compared to 13.6 ± 1.1 months in non-Hispanics (p = 0.011).ConclusionBlack and non-Hispanic patients have poorer cancer-specific survival in PMCTs.

Outcomes of Octogenarians with Primary Malignant Cardiac Tumors: National Cancer Database Analysis.

Data concerning age-related populations affected with primary malignant cardiac tumors (PMCTs) are still scarce. The aim of the current study was to analyze mortality differences amongst different age groups of patients with PMCTs, as reported by the National Cancer Database (NCDB). The NCDB was retrospectively reviewed for PMCTs from 2004 to 2017. The primary outcome was late mortality differences amongst different age categories (octogenarian, septuagenarian, younger age), while secondary outcomes included differences in treatment patterns and perioperative (30-day) mortality. A total of 736 patients were included, including 72 (9.8%) septuagenarians and 44 (5.98%) octogenarians. Angiosarcoma was the most prevalent PMCT. Surgery was performed in 432 (58.7%) patients (60.3%, 55.6%, and 40.9% in younger age, septuagenarian, and octogenarian, respectively, p = 0.04), with a corresponding 30-day mortality of 9.0% (7.0, 15.0, and 38.9% respectively, p < 0.001) and a median overall survival of 15.7 months (18.1, 8.7, and 4.5 months respectively). Using multivariable Cox regression, independent predictors of late mortality included octogenarian, governmental insurance, CDCC grade II/III, earlier year of diagnosis, angiosarcoma, stage III/IV, and absence of surgery/chemotherapy. With increasing age, patients presented a more significant comorbidity burden compared to younger ones and were treated more conservatively. Early and late survival outcomes progressively declined with advanced age.

Specific causes of death among patients with cardiac sarcoma in the United States-An analysis of The Surveillance, Epidemiology, and End Results (SEER) Program.

Representing 10%-25% of the primary cardiac neoplasms, primary malignant cardiac tumors (PMCT) have a poor prognosis, yet with rare incidence. Constituting more than 50% of the PMCT, cardiac sarcoma has increased not only in incidence over the past five decades but also in severity. Patients with PMCTs, especially sarcomas, have the worst prognosis when compared with other cardiac or extracardiac tumors. This retrospective study was performed using SEER*stat software, latest version 8.3.9.2(5) by accessing seer 18 registries plus data (excl AK) Nov 2020 Sub (2000-2018) for standard mortality ratio (SMR). A total of 235 patients were identified of whom 49.4% were females. Most of our patients were Stage 4 (n = 81) and distant in location (n = 92). The most common treatment method was tumor-directed surgery (n = 164), chemotherapy (n = 146), and radiotherapy (n = 55). Furthermore, other associated cancers with cardiac sarcoma were rare, with ten cases with lung and bronchus cancer (SMR: 37.95, 95% confidence interval [CI]: 18.2, 69.8), four with bone and joints (SMR: 1726.05, 95%CI: 470.2, 4419.3). During a 5-year follow-up, 235 patients died primarily due to cardiac sarcoma (n = 182, 77.4%), other cancers (n = 34, 14.4%), and other noncancerous causes (n = 19, 8%), while the noncancerous causes were attributed mainly to cardiovascular diseases (n = 4, 21%, SMR: 4.95, 95%CI: 1.35, 12.67), septicemia (n = 2, 10.5%, SMR: 41.23, 95%CI: 4.99, 148.95).

Lupus-like Syndrome and Cardiac Tamponade as Initial Presentation of a Primary Cardiac Malignant Tumor: Report of a Case

This is the case of a 38-year-old man with a history of two female relatives with Systemic Lupus Erythematosus and personal factors of high cardiovascular risk (gender, stress, smoking, chronic consumption of electronic cigarette). The patient presented tamponade due to pericardial effusion secondary to Lupus-like syndrome that preceded and accompanied at all times an angiosarcoma of the roof and anterior wall of the right atrium. His clinical evolution is described, as well as the surgical and pharmacological treatment in the Cardiovascular Care Unit of a third-level hospital and the possible causes of the poor response to management that led to his death.

L'imagerie en coupe dans le bilan des tumeurs intra-cardiaques

The etiology of cardiac masses is often oncological or thrombotic, rarely inflammatory. Among heart tumors, the vast majority are metastatic. We describe the most frequent benign primary cardiac tumors and the most frequent malignant primary cardiac tumors and give information about the advantages of using a multi-modality approach for the accurate diagnosis of a cardiac mass using Computed Tomography Scanner and Magnetic Resonance Investigation.

Primary Cardiac Sarcoma of the Left Atrium, A Case Report and Literature Review

Primary malignant cardiac tumours are extremely rare. They count for a quarter of all possible cardiac tumors, and are mostly sarcomas. For a long time, their diagnosis was made post mortem, and they were only reported through autopsy series. Since then, diagnostic and therapeutic advances have been made, making clinical diagnosis of this entity more accessible ante mortem. Nevertheless, due to their rarity, cardiac sarcomas suffer from a lack of therapeutic guidelines. Their treatment is modelled on that of sarcomas developed in other organs of the body. We report an interesting case of a 74-year-old female patient operated on for an infiltrating sarcomatous tumour of the left atrium revealed by dyspnoea associated with cardiac palpitations. Thoracic angioscan and transthoracic echocardiography led to the diagnosis of a cardiac tumour, and the thoracic-abdominal-pelvic Computed Tomography (CT) scan did not reveal any other extracardiac lesions except for a minimal non-neoplastic bilateral pleural effusion. The patient underwent an incomplete surgical procedure with R2 resection followed by adjuvant chemotherapy with doxorubicin monotherapy. The evolution under this treatment was marked by a locoregional progression of the disease with further progression in the atria, mediastinum and dorsal spine after 4 courses. A second line of chemotherapy with Dacarbazine monotherapy was initiated, but the patient died shortly after receiving two courses of the new treatment.

Trends in treatments and in-hospital outcomes of primary malignant cardiac tumors: A 16-year U.S. nationwide study.

e18756 Background: Primary malignant cardiac tumors are a rare but highly aggressive disease. Recent studies have quantified its long-term survival and the efficiencies of different treatment modalities. However, the trends in treatment patterns and in-hospital outcomes in the United States are still poorly understood. Methods: The National Inpatient Sample (NIS) was queried to identify adult patients (age ≥ 18) who were diagnosed with primary malignant cardiac tumors (ICD-9: 164.1; ICD-10: C38.0). Surgical treatments and chemotherapy were identified using the corresponding procedure and ICD codes. The national estimates were generated based on the weighted values of the hospitalizations. Results: From 2002 to 2017, 6,494 malignant cardiac tumor-related hospitalizations were identified. The mean age was 53.8 ± 17.5 years, 55.9% White, and 47.9% women. There was an increasing trend in the incidence over the study period, from 1.15 per 100,000 NIS admissions in 2002 to 1.49 per 100, 000 NIS admissions in 2017 (P for trend &lt; 0.001). 1708 (26.3%) patients received some forms of surgery: 733 (11.2%) underwent surgical excision of the tumor, 585 (9.0%) received pericardiotomy, and 397 (6.1%) underwent pericardiectomy. A decreasing trend in surgical therapy was observed (22.0% in 2002 to 16.8% in 2017, P for trend &lt; 0.001). 1403 (21.6%) patients received chemotherapy, with a significant decreasing trend over time (25.8% in 2002 to 19.6% in 2017, P for trend &lt; 0.001). The in-hospital mortality of the entire cohort was 11.1%, which remained unchanged over time (P for trend &gt; 0.05). Conclusions: This study demonstrates that although malignant cardiac tumor-related hospitalizations were increasing between 2002 and 2017, the uses of surgical therapy and chemotherapy were decreasing. The in-hospital mortality was high but remained stable over the study period.

Incidental finding of a primary cardiac leiomyosarcoma originating from the right atrium of a 25-year old male. A Case Report

BACKGROUND: Primary cardiac tumors are extremely rare and encompass less than 1% of all documented cardiac neoplasms with a majority of these being benign. Among the malignant primary cardiac tumors, leiomyosarcoma accounts for only 1% all documented cases and it commonly involves the left atrium. We present a rare case of a primary cardiac leiomyosarcoma originating from the right atrium of a 25-year-old male.CASE: The patient was a 25-year-old Filipino male who presented with progressive dyspnea, orthopnea, and a large, heterogenous, ovoid mass attached at the atrial septum measuring 7.0 cm x 3.16cm in widest dimension on 2D-echocardiography. The patient eventually underwent emergency excision of the right atrial mass. Post-operatively, the patient did not tolerate the procedure and ultimately expired after attempts of cardiopulmonary resuscitation.HISTOPATHOLOGIC FINDINGS: Microscopic sections show a spindle-cell neoplasm with irregular fascicles and whorled configurations interspersed with inflammatory cells in a fibromyxoid stroma. The spindle cells showed pleomorphic, hyperchromatic nuclei, some with prominent nucleoli and eosinophilic cytoplasm. Mitotic figures, areas of necrosis, and hemorrhages are seen. Immunohistochemical studies show reactivity of the neoplastic cells to Smooth Muscle Actin (SMA) and Vimentin. Masson’s Trichrome Stain is also positive. S100, Myogenin, and Desmin show non-reactivity. Proliferation index is at 15-20% using Ki-67 and p53.CONCLUSION: Primary cardiac leiomyosarcoma is an extremely rare tumor occurring in less than 1% of all documented primary malignant cardiac neoplasms. Metastases originating from other sites are more common. It has an aggressive clinical course and a dismal prognosis with features overlapping that of other soft tissue neoplasms. Hence, additional immunohistochemical studies and special stains should be employed to arrive with a definitive diagnosis so that appropriate management and intervention may be offered to affected patients.

Sex differences in primary malignant cardiac tumors: A multi-institutional cohort study from National Cancer Database.

Despite the significant clinical importance of sex among factors affecting cancer progression and survival, it remains one of the least studied factors. Therefore, we sought to examine these differences in relation to primary malignant cardiac tumors (PMCTs) using a national data set. The 2004-2017 National Cancer Database was queried for patients with PMCTs. Annual trend of females' percent was assessed. Overall survival predictors were evaluated with Kaplan-Meier and Cox-regression. Subgroup analysis was done based on histology, comorbidity index, race, insurance, and surgical treatment. PMCTs were identified in 736 patients (median age 52, female [47.8%]). Most of them were high-grade (49.2%). About 60% underwent surgery. Angiosarcoma (43%), fibrosarcoma (5.2%), and leiomyosarcoma (5.2%) were the most common pathologies. Based on multivariate Cox-regression, higher income, higher comorbidity index, angiosarcoma, and Stage III/IV were associated with higher late mortality, while year of diagnosis and use of surgery or chemotherapy were associated with lower mortality. Among the surgical group, age, higher income, higher comorbidity index, angiosarcoma, and Stage III/IV were independent predictors of higher late mortality, while private insurance and year of diagnosis were associated with lower late mortality. No difference was seen between males and females in 30-day and late mortality (p = .71). Subgroup analysis based on Cox-regression showed no differences in late mortality between males and females. PMCTs have poor overall survival. Surgery and chemotherapy were associated with longer survival benefits. On the contrary, the associated risk factors for mortality were advanced age, higher comorbidity index, angiosarcoma histology, and Stage III/IV.