Primary Malignant Cardiac Tumors Research Articles

1113 Primary and secondary malignant cardiac tumors - a 22 year case review

Abstract Background Primary cardiac tumors are extremely rare and are usually diagnosed late due to the non-specific symptomatology. Surgery is the main treatment option and despite chemotherapy, the prognosis remains poor. Cardiac invasion by metastatic tumors, while more common, also entails an unsatisfactory outcome. Purpose To review patients (P) with malignant cardiac tumours that were diagnosed by transthoracic echocardiography (TTE) or by transesophageal echocardiogram (TEE) in a tertiary center between 1997 and 2019. Methods Retrospective analysis of clinical data from the digital files, echocardiographic assessment of tumor location and morphology, histology results and survival outcomes. Results A total of 33 malignant cardiac tumors were diagnosed: 12 primary tumors (A) and 21 metastatic tumors (B). A Regarding primary cardiac tumors, the most common types were angiosarcomas (6 cases), 2 undifferentiated pleomorphic sarcomas, 1 right ventricle (RV) sarcoma, 1 primary cardiac lymphoma, 1 myxofibrosarcoma and 1 fibrosarcoma. The mean age of the P at time of diagnosis was 43 ± 15 years, 50% female gender. The most frequent presentation was heart failure symptoms (50% of P) followed by arrhythmias (20%). One patient had a rare presentation with pruritus and polyarthralgias. On TTE, the most prevalent tumor location was in the right-heart chambers (70%) - mostly the right atrium (50%), with mean dimensions of 40 ± 18 mm x 27 ± 11 mm. 85% of patients had preserved systolic left ventricular function and there was moderate or severe pericardial effusion in 38%. The most frequent distant metastatic involvement of primary tumors at diagnosis was pulmonary (33%) and hepatic (33%). 50% of P were submitted to tumor resection and 40% were submitted to chemotherapy. In the case of angiosarcomas, the most common immunohistochemical markers were vimentin, CD31 and CD34. The authors found a mortality rate of 81% in P with primary cardiac tumors, with a median time of follow-up of 6 months (minimum of 20 days and maximum of 18 years). In the latter case, the P was submitted to heart transplantation after diagnosis of a sarcoma of the RV and is still alive and well. B Regarding secondary cardiac invasion, there was a diagnosis of the following primary tumor sites: 6 thymomas, 4 cases of lymphoma, 3 lung carcinomas, 3 hepatocellular carcinomas, 2 bladder carcinomas, 1 parathyroid carcinoma, 1 soft tissue sarcoma and 1 melanoma. The mean age of P with metastastic involvement of the heart was 57 ± 22 years, 65% male. On TTE/TEE the authors also found a right-sided chambers predominance (60%), with pericardial metastasis in 35%. As expected, the mortality rate was also extremely high (90%), with a median time of follow up of 1.5 months (minimum of one week, maximum of 44 months) Conclusion Cardiac malignant tumors generally present in a late stage with a dismal prognosis. When possible, heart transplantation can be an option with a good outcome.

Right atrial and SVC infiltrating mass-the entity of infiltrating lipoma

BackgroundCardiac lipomas are rare benign primary cardiac tumours primarily composed of mature adipocytes. They are usually well defined, encapsulated masses, but rarely demonstrate malignant characteristics by infiltrating the myocardium. This causes diagnostic uncertainty as it becomes a priority to rule out primary malignant cardiac tumours such as sarcoma which often carry a poor prognosis.Case ReportA 61 year old female presenting with chest pain was found to have an infiltrating right atrial hypertrophic mass. Mutli-disciplinary team (MDT) discussions along with the presence of symptoms and likelihood of malignancy led to the recommendations for surgery.Intraoperatively this involved the right pulmonary veins and superior vena cava (SVC). The mass was resected with good margins and reconstruction of the right atrium, pulmonary veins and SVC was done using porcine pericardial patch. The patient made a good postoperative recovery and was discharged home in sinus rhythm with no significant valvular lesions. This was further confirmed at 6 month follow up. Final histology was that of infiltrating lipoma.ConclusionsIn this rare case of infiltrating cardiac lipoma in a relatively young patient, the diagnostic uncertainty despite multimodal imaging meant surgery was indicated due to the high suspicion of cancer. Even in benign cases, fatty infiltration can lead to conduction defects and embolisation. Technical difficulties in sectioning these specimens is caused by intra-tumour variability and current recommendations are for excision biopsy, for best characterisation.The management of these patients requires an MDT with Cardiac surgery being a safe approach providing definitive management.

Nomograms and risk classification systems predicting overall and cancer-specific survival in primary malignant cardiac tumor.

Primary malignant cardiac tumors (PMCTs) are fatal, but up to now, there is still a lack of survival prediction model for prognosis evaluation. We developed nomograms to predict overall survival (OS) and cancer-specific survival (CSS) for PMCTs by the Surveillance, Epidemiology, and End Result (SEER) database. A total of 506 PMCTs participants were identified in the SEER database from 1973 to 2014 and were randomly assigned into the training cohort (N = 354) and the validation cohort (N = 152). The prognostic factors for PMCTs were identified by Kaplan-Meier and multivariate Cox analysis and further incorporated to build OS and CSS nomograms. The nomograms were internally and externally validated via concordance indexes (C-index) and calibration curves. The independent prognostic factors for OS and CSS in PMCTs were associated with age at diagnosis, histopathology, tumor stage, cancer-directed surgery, and chemotherapy (all P < .05). In the internal validation, the C-index values were 0.71 (95% confidence interval [CI]: 0.68-0.75) for OS nomogram, and 0.70 (95% CI: 0.67-0.74) for CSS nomogram. In the external validation, the C-index values were 0.71 (95% CI: 0.66-0.77) for OS nomogram, and 0.71 (95% CI: 0.65-0.77) for CSS nomogram. The calibration curves of internal and external validation showed consistency between the nomograms and the actual observation. The risk stratification of PMCTs was significant distinction (P < .05). We developed and validated credible nomograms to predict OS and CSS in PMCTs. These nomograms can be offered to clinicians to more precisely estimate the survival and identify risk stratification of PMCTs.

Primary Malignant Cardiac Tumors: A Nationwide Analysis of Incidence, Treatment Factors, and Outcome

Primary Malignant Cardiac Tumors: A Nationwide Analysis of Incidence, Treatment Factors, and Outcome

Outcome and Pathological Characteristics of Primary Malignant Cardiac Tumors.

The literature on malignant cardiac tumors is relatively limited because they are rare, especially among the Chinese population. We analyzed 14 patients diagnosed with malignant cardiac tumors in Fuwai Hospital and present the results of surgical treatments on the tumors. The mean age at tumor diagnosis was 47 years in a male-dominated cohort. There was a high frequency of pericardial effusion and coronary artery involvement in our group. We compared the survival times of patients who received different treatments and found that surgery improved prognosis of tumors, especially for patients who underwent orthotopic heart transplantation.

Primary Cardiac Angiosarcoma: Authors' Perspective Following Synthesis of Available Literature

Why are malignant primary cardiac tumors so rare? Is it because of the specialized cardiac muscle cells or the large number of mitochondria per cell? Is it because cardiac muscle is the most aerobic organ of the body and its myocardial capillary density is at a 1:1 ratio, which is 10-fold higher than that of the skeletal muscle? There is overwhelming evidence regarding the benefits of aerobic exercise in prevention of many types of cancer and their associated sequelae. However, there appears to be an evidence gap that needs to be filled. Is there a link between the rare occurrence of primary cardiac cancers in terms of the oxygenation of cardiac muscle tissue and the benefits of aerobic exercise that could provide a new area of research for the primary prevention of cancer? Discussion of these questions was the impetus for the authors to conduct a literature search and then synthesize the review findings. This perspective aims to explore currently available knowledge and gaps in available evidence to understand areas in which physical therapists may impact the varied levels of preventive intervention on primary malignant cardiac cancers. The 35 presenting symptoms and medical history reported in the literature may provide a basis for correlation with other genetic and epigenetic factors to explore along our journey for answers. The physical therapy profession is well positioned to promote healthy behaviors and provide patient education to reduce risk factors and prevent and treat noncommunicable diseases consistent with a biopsychosocial paradigm.

Primary Malignant Cardiac Tumors (PMCTs) Successful Resection of a Huge Liposarcoma of the Heart

Primary Malignant Cardiac Tumors (PMCTs) Successful Resection of a Huge Liposarcoma of the Heart

Primary Malignant Cardiac Tumors (PMCTs) Successful Resection of a Huge Liposarcoma of the Heart

Primary cardiac tumors are rare with an incidence ranging from 0.001% to 0.03% in autopsy series. The prognosis of cardiac sarcomas remains poor because it proliferates rapidly, and distant metastases are often found at diagnosis. We present a case of liposarcoma in the atrium of the heart as case report and same time ,we do a literature review about it. Case Report: We present the case of a young 30 years old female with a significant tumor of cardiac liposarcoma, with chief complaint of dyspnea, tachycardia and heart failure even angina pectoris. Conclusion: The diagnostician’s differential diagnosis must be broad when encountering common chief complaints, such as tachycardia, heart failure and angina pectoris.

Risk factors for early death in primary malignant cardiac tumors: An analysis of over 40 years and 500 patients

ObjectivesTo investigate risk factors contributing to early death in patients diagnosed with primary malignant cardiac tumors (PMCTs) and derive better understanding of these poorly characterized individuals. MethodData from the Surveillance, Epidemiology and End-Results (SEER) registries on 564 patients diagnosed with PMCTs between 1973 and 2014 were analyzed. Early death was defined as survival of ≤3 months from the time of diagnosis. Two-tailed χ2 or fisher's exact test were used for association between categorical variables and occurrence of early death. Logistic regression analysis was used to assess independent risk factors of early death. Time trends in early death rates of PMCTs were described using scatter plot. ResultsOf the 564 patients with PMCTs, early death was identified in 214 individuals (37.9%). Patients with unspecified soft tissue sarcomas and blood vessel tumors had the highest risk of early death. Age > 80 years and non-consent for surgery were strong predictors of early death in all PMCT subtypes. In sarcomas, disadvantaged income was associated with an increase in early mortality, while black race was associated with a reduction in early mortality. In mesotheliomas and others, male sex was a risk factor for early mortality, while Hispanic ethnicity was associated with a reduction in early mortality. Percentages of early death slightly decreased over the past 40 years. ConclusionsPredictors of early death are primarily related to age older than 80 years, no surgery and specific histopathology types but also include disadvantaged socioeconomic status and male sex. Initiatives to identify those at risk and develop preventive interventions should be prioritized.

Comprehensive review of the epidemiology and treatments for malignant adult cardiac tumors.

Carcinomas rarely occur in the heart, and cardiac surgeons are generally not familiar with cardiac tumors. Some characteristics of cardiac and intrapericardial tumors are reviewed to understand the features of cardiac tumors. Cardiac tumors are discussed separately from intrapericardial tumors. Primary cardiac tumors are predominantly benign whereas primary intrapericardial tumors are usually malignant. The prevalence of each tumor types is presented in this review. In both cardiac and intrapericardial tumors, the incidences of metastatic tumors from cancers outside of the heart are high with carcinomas occupying more than half of the cases. Generally, the prognosis of primary malignant cardiac tumors is very poor. Cardiac tumors metastasize to other organs as hematogenous metastases. Surgery must be performed based on all the above-mentioned features of both cardiac and intrapericardial tumors.

Predictive value of the metabolic tumor volume ratio of cardiac mass to maximal extra cardiac mass for the origin of cardiac tumors

Objective To explore the value of metabolic volume of cardiac tumor (MTV1) to that of the maximum extracardiac tumor (MTV2) ratio in predicating the cardiac tumor origin. Methods A total of 35 consecutive cases (19 males, 16 females, age range: 18-68 years) with multiple cardiac and extracardiac tumors were enrolled in this retrospective analysis. All of them were confirmed by pathology or clinical follow-up results and examined by 18F-fluorodeoxyglucose PET/CT from January 2010 to February 2016. Maximum standardized uptake value (SUVmax) 3.63 was used as the background threshold. MTV1 and MTV2 were automatically obtained by PETVCRA software. Receiver operating characteristic (ROC) curve was drawn to obtain the diagnostic threshold of MTV1/MTV2 ratio for cardiac tumors, and the sensitivity, specificity and accuracy were calculated. Mann-Whitney u test was used to analyze the data. Results Twelve patients were confirmed to have primary cardiac malignant tumors (PCMT), and 23 cases were metastatic cardiac malignant tumors (MCMT). There was statistical difference of MTV1 between PCMT and MCMT patients: 52.9(33.3, 703.4) cm3vs 8.1(1.2, 24.6) cm3 (z=-3.70, P<0.05). MTV2 was 11.7(1.8, 38.4) cm3 in PCMT patients, which was lower than that in MCMT patients (182.0(100.1, 238.0) cm3;z=-4.17, P<0.05). MTV1/MTV2 ratio of PCMT was 16.20(9.40, 71.80), which was significantly higher than that of MCMT (0.10(0.01, 0.60), z=-4.66, P<0.05). When MTV1/MTV2 ratio=1.2 was selected as the cut-off value, the sensitivity, specificity and accuracy were 12/12, 91.30%(21/23), 94.29%(33/35) respectively. Conclusion It may be an important criterion for the diagnosis of PCMT that MTV1 is greater than MTV2. Key words: Heart neoplasms; Positron-emission tomography; Tomography, X-ray computed; Deoxyglucose

Surgical Treatment of Cardiac Tumors: Insights from an 18-Year Single-Center Analysis.

BackgroundThe aim of this study was to investigate the clinical presentation, operative data, and early and late outcomes of a large patient cohort undergoing surgical treatment for cardiac tumors in our institution.Material/MethodsA total of 181 patients underwent surgery because of suspected cardiac tumor in our institution between 1998 and 2016. In 162 cases, the diagnosis was confirmed postoperatively and these patients were included in this study. Preoperative baseline characteristics, operative data, and postoperative early and long-term outcomes were analyzed.ResultsMean age at presentation was 56.6±17.6 years, and 95 (58.6%) patients were female. There were 126 (77.8%) patients with benign cardiac tumors, while the remaining patients had malignant tumors (primary and metastasized). The mean follow-up time was 5.2±4.7 years. The most frequent histologically verified tumor type was myxoma (63%, n=102). In terms of malignant tumors, various types of sarcomas presented most primary malignant cardiac tumors (7.4%, n=12). The mean ICU length of stay was 1.7±2.2 days and overall in-hospital mortality was 3.1% (n=5). Frequent postoperative complications included mediastinal bleeding (5.8%, n=9), wound infection (1.3%, n=2), acute renal failure (5.6%, n=9), and major cerebrovascular events (n=7, 4.6%). The overall cumulative survival after cardiac tumor resection was 94% at 30 days, 85% at 1 year, 72% at 5 years, and 59% at 15 years.ConclusionsSurgical treatment of cardiac tumors is a safe and highly effective strategy associated with good early and long-term outcomes.

P6302Predictors of survival in primary malignant cardiac tumors: a seer-based analysis

P6302Predictors of survival in primary malignant cardiac tumors: a seer-based analysis

Postoperative Complications and Long-Term Results after Primary Cardiac Sarcoma Resection.

Primary malignant cardiac tumors rarely occur in clinical care. Little is known about the impact of a parameter on postoperative survival. From May 1991 to May 2014, a total of 24 patients underwent surgical treatment of a primary cardiac sarcoma in our center. We analyzed our clinical database retrospectively for information on patient characteristics and treatment data. The follow-up could be completed to 91.7%. Angiosarcoma and non-otherwise-specified sarcoma were the most common tumor entities. R0 resection was achieved in most cases. Postoperative mortality within the first 30 days was 20.8% (n = 5). In four of these five cases, postoperative low-output cardiac failure was the leading cause of death. The cumulative survival rate was 77.3% after 30 days, 68.2% after 3 months, 50.0% after 6 months, 45.0% after 12 months, and 18.0% after 24 months. The mean survival time in the whole group was 47.0 months. A low tumor differentiation was associated with low mean survival, but this was not statistically significant. Mean survival of sarcoma was higher after R0 resection. There was no significant rate of survival difference regarding the adjuvant therapy concept. Extended surgery alone or in combination with chemo- and/or radiotherapy may be successful in certain cases and may offer a satisfactory quality of life. The establishment of a multicenter heart tumor register in Germany is necessary to increase the number of cases in studies, get more remarkable study results, and standardize the diagnosis and therapy.

Prevalence of primary cardiac tumor malignancies in retrospective studies over six decades: a systematic review and meta-analysis.

The incidence of patients diagnosed with primary malignant cardiac tumors (PMCTs) has increased greatly in the past few decades. Whether this rising prevalence is due to overdiagnosis or an increased malignancy rate of primary cardiac tumors (PCTs) remains unclear. Therefore, we performed a systematic review and meta-analysis of published retrospective studies to determine whether the malignancy rate has been increasing over time. Published studies containing relevant data between 1956 and 2014 were evaluated. Two authors searched for all retrospective studies that included patients diagnosed with PCT and PMCT. Two other investigators independently extracted the data, and discrepancies were resolved by consensus. A random-effects meta-analysis model and cumulative meta-analysis model were used to evaluate the pooled prevalence and trend of dynamic change in PCT malignancies. The effects of time, study period and sample size were studied using a logit-linear regression model with robust error variance and a time variable. Thirty-eight studies involving 5,586 patients were analyzed. The pooled prevalence of PMCT among the patients diagnosed with PCT was 9.9% (95% CI, 8.4% to 11.4%) (I2=70%; P< 0.001), and this prevalence has been stable since around 2003. In the regression model, the malignancy odds ratio remained stable from 1975 onward, and no time effect was observed. Our study confirms that PMCT is uncommon, and the prevalence of PCT malignancies remained stable in the past few decades. The clinically observed increase in incidence is unlikely to reflect a true population-level increase in tumorigenesis. This result strongly suggests that the observed increase in incidence of PMCT most likely reflects increased diagnostic detection over time.

Cardiac Magnetic Resonance Imaging in Oncology.

Cardiac magnetic resonance imaging (MRI) is emerging as an important diagnostic modality in the management of cardiovascular-related dysfunction in oncological diseases. Advances in imaging techniques have enhanced the detection and evaluation of cardiac masses; meanwhile, innovative applications have created a growing role for cardiac MRI for the management of cardiotoxicity caused by cancer therapies. An overview is provided of the clinical indications and technical considerations of cardiac MRI. Its role in the evaluation of cardiac masses and cardiac function is reviewed, and novel sequences are discussed that are giving rise to future directions in cardio-oncology research. A review of the literature was also performed, focusing on cardiac MRI findings associated with cardiac dysfunction related to cancer treatment. Cardiac MRI can be used to differentiate benign and malignant primary cardiac tumors, metastatic disease, and pseudotumors with high spatial and temporal resolution. Cardiac MRI can also be used to detect the early and long-term effects of cardiotoxicity related to cancer therapy. This is accomplished through a multiparametric approach that uses conventional bright blood, dark blood, and postcontrast sequences while also considering the applicability of newer T1 and T2 mapping sequences and other emerging techniques. Cardio-oncology programs have an expanding presence in the multidisciplinary approach of cancer care. Consequently, knowledge of cardiac MRI and its potential applications is critical to the success of contemporary cancer diagnostics and cancer management.

Primary Malignant Cardiac Tumor Presenting with Dyspnea: A Case Report

Primary Malignant Cardiac Tumor Presenting with Dyspnea: A Case Report

Primary malignant cardiac tumors: Surgical results

Primary malignant cardiac tumors: Surgical results

Primary malignant cardiac tumors: Surgical results

ObjectiveTo characterize primary malignant cardiac tumors operated on in our center and to analyze patient survival. MethodsBetween January 1994 and August 2014, 123 patients with cardiac tumors underwent surgery, of which 12 (9.8%) were primary malignant tumors – eight sarcomas (67%), three B-cell lymphomas (25%) and one epithelioid hemangioendothelioma (8.3%). The tumor affected the left atrium in five cases (42%), the right atrium in four (33%), the right ventricle in two (17%) and the pulmonary valve in one (8%). Patients’ mean age was 55.4±16.9 years, 67% were female and 75% presented in NYHA class III–IV. ResultsResection was complete (negative margins) in five cases and partial in seven (five sarcomas and two lymphomas), and 11 patients needed adjuvant therapy, surgery alone being curative in only one (epithelioid hemangioendothelioma). Mean follow-up was 41.7±61.3 months: 24.8±30.0 months (3.8–95.7) for sarcomas, 70.1±118.0 months (1–206.3) for lymphomas and 91.9 months for the epithelioid hemangioendothelioma. During follow-up, 10 patients died (83%) and two were alive (17%). Overall survival at 30 days, six months, one year and two years was 91.7%, 66.7%, 58.3% and 41.7%, respectively. In the sarcoma group, 1-year and 2-year survival were 62.5% and 37.5%, respectively. ConclusionsResection of primary malignant cardiac tumors, even partial, is safe, provides relief of obstructive symptoms and improves quality of life, but is rarely curative and has a low survival rate. Due to the rarity of such tumors, a multicenter database could improve knowledge and help clarify the indications for cardiac surgery as a treatment option.

Tumores cardíacos primários malignos: resultados cirúrgicos

ObjectiveTo characterize primary malignant cardiac tumors operated on in our center and to analyze patient survival. MethodsBetween January 1994 and August 2014, 123 patients with cardiac tumors underwent surgery, of which 12 (9.8%) were primary malignant tumors – eight sarcomas (67%), three B‐cell lymphomas (25%) and one epithelioid hemangioendothelioma (8.3%). The tumor affected the left atrium in five cases (42%), the right atrium in four (33%), the right ventricle in two (17%) and the pulmonary valve in one (8%). Patients’ mean age was 55.4±16.9 years, 67% were female and 75% presented in New York Heart Association class III–IV. ResultsResection was complete (negative margins) in five cases and partial in seven (five sarcomas and two lymphomas), and 11 patients needed adjuvant therapy, surgery alone being curative in only one (epithelioid hemangioendothelioma). Mean follow‐up was 41.7±61.3 months: 24.8±30.0 months (3.8–95.7) for sarcomas, 70.1±118.0 months (1–206.3) for lymphomas and 91.9 months for the epithelioid hemangioendothelioma. During follow‐up, 10 patients died (83%) and two were alive (17%). Overall survival at 30 days, six months, one year and two years was 91.7%, 66.7%, 58.3% and 41.7%, respectively. In the sarcoma group, 1‐year and 2‐year survival were 62.5% and 37.5%, respectively. ConclusionsResection of primary malignant cardiac tumors, even partial, is safe, provides relief of obstructive symptoms and improves quality of life, but is rarely curative and has a low survival rate. Due to the rarity of such tumors, a multicenter database could improve knowledge and help clarify the indications for cardiac surgery as a treatment option.