Cardiac Metastases in Patients with Neuroendocrine Tumours: Clinical Features, Therapy Outcomes, and Prognostic Implications.

BACKGROUND: Cardiac metastases (CM) from neuroendocrine tumours (NET) are rare; however, with the introduction of new molecular imaging modalities, such as 68Ga-DOTATATE PET-CT for NET diagnosis and re-staging, they are now identified more frequently. This study presents a single-institution experience on the NET CM characteristics, management, and prognostic implications. METHODS: Between January 1998 and January 2020, 25 NET patients with CM were treated in our unit. A retrospective review of electronic records was performed. Overall survival (OS) was assessed by the Kaplan-Meier method. Cox regression models were used to evaluate the association of various clinical variables with OS. RESULTS: The median age in the NET CM cohort was 64 years, with small intestine being the most common primary (84%). Nearly half of the patients suffered either from shortness of breath (48%) or had palpitations (12%). Peptide receptor radionuclide therapy (PRRT) was applied in more than half of the patients (64%), who had an improved trend for a longer median OS compared to those patients who did not receive PRRT (76.0 vs. 14.0 months, p = 0.196). The multivariate analysis demonstrated that concomitant skeletal or pancreatic metastases, as well as N-terminal pro-B-type natriuretic peptide (NT pro-BNP) >2 × upper limit of normal (ULN), were independent poor prognosticators. CONCLUSIONS: Clinical features of NET CM ranged from asymptomatic patients to heart failure. Concomitant bone or pancreatic metastases and NT pro-BNP levels >2 ULN predicted shorter survival time. PRRT serves as a feasible therapy with promising survival benefits; however, more data are needed.

Risk stratification of cardiac metastases using late gadolinium enhancement cardiovascular magnetic resonance: prognostic impact of hypo-enhancement evidenced tumor avascularity.

BACKGROUND: Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) is widely used to identify cardiac neoplasms, for which diagnosis is predicated on enhancement stemming from lesion vascularity: Impact of contrast-enhancement pattern on clinical outcomes is unknown. The objective of this study was to determine whether cardiac metastasis (CMET) enhancement pattern on LGE-CMR impacts prognosis, with focus on heterogeneous lesion enhancement as a marker of tumor avascularity. METHODS: Advanced (stage IV) systemic cancer patients with and without CMET matched (1:1) by cancer etiology underwent a standardized CMR protocol. CMET was identified via established LGE-CMR criteria based on lesion enhancement; enhancement pattern was further classified as heterogeneous (enhancing and non-enhancing components) or diffuse and assessed via quantitative (contrast-to-noise ratio (CNR); signal-to-noise ratio (SNR)) analyses. Embolic events and mortality were tested in relation to lesion location and contrast-enhancement pattern. RESULTS: 224 patients were studied, including 112 patients with CMET and unaffected (CMET -) controls matched for systemic cancer etiology/stage. CMET enhancement pattern varied (53% heterogeneous, 47% diffuse). Quantitative analyses were consistent with lesion classification; CNR was higher and SNR lower in heterogeneously enhancing CMET (p < 0.001)-paralleled by larger size based on linear dimensions (p < 0.05). Contrast-enhancement pattern did not vary based on lesion location (p = NS). Embolic events were similar between patients with diffuse and heterogeneous lesions (p = NS) but varied by location: Patients with right-sided lesions had threefold more pulmonary emboli (20% vs. 6%, p = 0.02); those with left-sided lesions had lower rates equivalent to controls (4% vs. 5%, p = 1.00). Mortality was higher among patients with CMET (hazard ratio [HR] = 1.64 [CI 1.17-2.29], p = 0.004) compared to controls, but varied by contrast-enhancement pattern: Diffusely enhancing CMET had equivalent mortality to controls (p = 0.21) whereas prognosis was worse with heterogeneous CMET (p = 0.005) and more strongly predicted by heterogeneous enhancement (HR = 1.97 [CI 1.23-3.15], p = 0.005) than lesion size (HR = 1.11 per 10 cm [CI 0.53-2.33], p = 0.79). CONCLUSIONS: Contrast-enhancement pattern and location of CMET on CMR impacts prognosis. Embolic events vary by CMET location, with likelihood of PE greatest with right-sided lesions. Heterogeneous enhancement-a marker of tumor avascularity on LGE-CMR-is a novel marker of increased mortality risk.

The incidence and survival outcomes of patients with primary cardiac lymphoma: A SEER-based analysis.

This study aimed to evaluate the incidence and prognosis of primary cardiac lymphoma (PCL) by using the Surveillance, Epidemiology, and End Results Program (SEER) database. Patients diagnosed with PCL and the disease incidence in the SEER database from 1975 to 2016 were included. Overall survival (OS) and cause-specific survival (CSS) curves were calculated using the Kaplan-Meier method and compared by the log-rank test. Univariate and multivariable Cox proportional hazard regression analyses were used to identify associations with outcome measures. The incidence of PCL was 0.011/100 000, and a predominance of elderly and male patients was observed. A total of 144 patients were enrolled. The median age of onset was 68 (9-96) years, including 80 (55.6%) males and 64 (44.4%) females. Multivariate analysis revealed that age and chemotherapy were independent prognostic factors for OS (both P < .05). Ann Arbor stage and chemotherapy were independent prognostic factors for CSS (both P < .05). In terms of treatment modality, chemotherapy combined with surgery was an independent protective factor for OS and CSS (both P < .05). For patients with primary cardiac diffuse large B-cell lymphoma (cardiac DLBCL), multivariate analysis also showed that age, Ann Arbor stage, and chemotherapy were all independent prognostic factors for OS and CSS (all P < .05). Chemotherapy combined with surgery was associated with a significant benefit in terms of OS and CSS (both P < .05). Our study confirmed that older age and advanced Ann Arbor stage were independent risk factors for PCL, and treatment with chemotherapy or cooperation with surgery resulted in better long-term survival.

Surgical Treatment of Cardiac Metastases: Analysis of a 13-Year Single-Center Experience.

BACKGROUND: Cardiac metastases are more common than primary malignant tumors of the heart and are usually treated surgically as a palliative approach. In this study, we reviewed our experience with the surgical treatment of patients with cardiac metastases of various types of malignant tumors. METHODS: Between 2003 and 2016, 10 patients underwent surgery for cardiac metastases at our institution. RESULTS: The mean age was 53.5 ± 19 years. Female patients made up 60% (n = 6) of the collective. The cohort included cardiac metastases of diverse origins (peripheral sarcomas, melanoma, rectal carcinoma, and hepatocellular carcinoma). The left side of the heart was more frequently affected (n = 7). In only six patients, the primary malignancy was known at the time of cardiac surgery. The interval between the first diagnosis of the primary tumor and cardiac metastases ranged from simultaneous diagnosis to up to 19 years. At the time of the diagnosis of the cardiac metastases, seven patients already had multiple metastases: all seven patients had pulmonary metastases, and three of them additionally had hepatic, cerebral, or osseous metastases. Only four patients were symptomatic (atrial fibrillation, pericardial effusion, tachycardia with chest pain, dyspnea). There was no in-hospital death. The postoperative course was uneventful overall. The one- and two-year survival rates were similar, that is, 76%. The median follow-up time was 5.4 years. CONCLUSIONS: Surgical intervention for treating cardiac metastases is associated with uneventful clinical outcome and acceptable survival in this critically ill population. Control of the primary malignancy, and maybe other metastases, determines the survival.

Prognostic Factors for Long-Term Survival after Surgical Resection of Primary Cardiac Sarcoma.

BACKGROUND: Primary cardiac sarcoma (CS) is an extremely rare disease. This study aims to identify possible prognostic factors for long-term survival. METHODS: A total of 17 consecutive patients who were treated for primary CS between 2003 und 2018 at two cardiac centers were investigated. Clinical data and histological characteristics of the tumors were analyzed. Long-term follow-up of all patients were performed. RESULTS: The median age was 54 years (range: 23-74). The tumors originated from the left side of the heart in nine patients. Histologically, there were four angiosarcomas, three intimal sarcomas, and three synovial sarcomas. One- and 7-year survivals were 81.9 and 18.2%, respectively. Low expression levels of Ki-67 tended to be associated with increased survival (log-rank p = 0.06). Adjuvant chemotherapy but not radiotherapy regardless of existing metastases was associated with significantly increased survival (log-rank p = 0.001). CONCLUSION: Angiosarcoma was the most common type of CS. The survival of CS patients is poor but prognostic factors, such as Ki-67, may help estimate the course of the disease. Survival could be improved significantly with chemotherapy.

Primary cardiac synovial sarcoma: A review correlating outcomes with surgery and adjuvant therapy.

BACKGROUND: Cardiac synovial sarcoma (CSS) is an extremely rare malignant tumor with a severe prognosis, due to frequent relapses and metastases. To obtain useful information for treatment protocols, we analyzed survival and therapy data from the cases reported in the literature. METHODS: A search of MEDLINE was performed throughout December 2018. Using key words relating to primary CSS, we collected from the literature a total of 97 cases, mainly consisting of single case reports. To identify predictors of overall survival, statistical analyses were performed on a selected cohort of 55 patients for whom relevant clinicopathological data were available, including surgery and adjuvant therapy. RESULTS: The univariable analysis revealed that patients in their first three decades of life have better overall survival. The univariable analysis also showed that patients not receiving adjuvant chemotherapy are at increased risk of death. In the multivariable analysis, tumor resection and chemotherapy are factors significantly improving overall survival. CONCLUSION: The survival of patients with CSS is positively influenced by a young patient's age and greatly improved by the administration of chemotherapy, even in the absence of tumor resection.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Survival of Patients with Primary Cardiac Tumors in an 11-Year Single Center Study.

BACKGROUND: Primary cardiac tumors are rare, but few studies have examined the relationship between risk factors and the prognosis. The aim of this study was to provide a survival analysis and risk factors for mortality in patients with primary cardiac tumors. METHODS: We retrospectively enrolled 71 patients diagnosed with primary cardiac tumors from June 2006 to November 2017 in our hospital. Patients' population characteristics, treatment information, pathology, and follow-up data were obtained and analyzed. RESULTS: Of the 71 patients, 60 cases were benign, and 11 cases were malignant. Sex, age, New York Heart Association classification, the percentage of peripheral embolism, and surgery had no significant difference between benign and malignant groups (P >.05), but the percentage of arrhythmia, leg edema, and mortality rate was higher in the malignant tumor group than in the benign tumor group (P <.05). Compared with the benign tumor group, the percentage of biatrial lesions in the malignant tumor group was significantly higher (P <.05). Moreover, Independent risk factors included the treatment choice, pathology type, and number of tumor lesions (P <.05). CONCLUSION: Our study suggests that conservative therapy, malignant cardiac tumor, and biatrial tumor lesion are independent risk factors for poor prognosis.

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 myxofibrosarcoma: case report, literature review and pooled analysis.

BACKGROUND: Primary cardiac myxofibrosarcoma is a very rare cardiac malignancy. The majority of publications are limited to case reports. No pooled analyses of primary cardiac myxofibrosarcoma cases are available. Little clinical features and outcome patterns are acknowledged. The purpose of this study is to identify the clinical characteristics and prognostic factors of primary cardiac myxofibrosarcoma. CASE PRESENTATION: A case report of primary cardiac myxofibrosarcoma was presented, and a review of English language literatures of primary cardiac myxofibrosarcomas were performed electronically. Demographics, clinicopathologic data, therapy and follow-up were summarized. The median survival time and the mean survival time were calculated by Kaplan-Meier method. Survival distribution and overall survival were figured by log-rank test and cox proportional hazards models. We present a case, and retrospectively analyzed additional 30 patients derived from 24 isolated articles. The cohort consisted of 18 male and 13 female patients. The age was 41.87 ± 17.89 years. Some common features were found in clinical presentations, pathologic features, treatments and outcome patterns of primary cardiac myxofibrosarcoma. There were special features in echocardiography, histological and immunohistochemical examinations, which should be considered in diagnosis of primary cardiac myxofibrosarcoma. The median survival time/mean survival time (MST) was 14/32.66 months. The median survival time/mean survival time (MST) was 14/32.66 months. Compared to the other groups, the following groups had shorter survival characteristics, including age ≥ 40 years (14/11.79 months), female (14/26.26 months), mass diameter ≥ 40 mm (14/14.64 months), high-grade (2/11.81 months), and no post-treatment (14/28.09 months). Statistical analyses revealed that primary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases. Tumors ≥ 40 mm in size (P = 0.055, HR = 6.79) or with high-grade (P = 0.063, HR = 11.45) had significantly worse prognosis. CONCLUSIONS: Primary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases. Echocardiography, together with histological method should be considered in ordinary diagnosis. Tumors ≥ 40 mm in size or with high-grade had significantly worse prognosis, which should be early diagnosed and treated with rational surgery.

Echocardiographic characteristics of primary malignant pericardial mesothelioma and outcomes analysis: a retrospective study.

BACKGROUND: Little is known about the echocardiographic characteristics of primary malignant pericardial mesothelioma (PPM) due to its rarity. The aim of this study was to explore the sex-specific echocardiographic patterns of PPM and risk factors for in-hospital mortality. METHODS: A retrospective information retrieval was conducted for cases of PPM reported from China during 1981 and 2015. The diagnosis was made by histopathological examinations and only cases with echocardiographic descriptions were included. Data on the clinical and echocardiographic findings were collected. Difference in clinical, sex-specific echocardiographic characteristics and findings across different time periods were assessed. Logistic regression analysis was performed to explore echocardiographic risk factors for in-hospital mortality. RESULTS: A total of 64 patients with PPM were included, with a mean age of 39.2 ± 15.6 years and minor male dominance (40, 62.5%). The most common echocardiographic presentations were pericardial effusion (55, 85.9%), pericardial masses (36.4%) and thickening (17.3%), respectively. The positive rate of pericardiocentesis was only 20.9%. Six patients (15.4%) died among 39 cases reporting in-hospital outcome. Logistics analysis identified no clinical or echocardiographic parameters associated with in-hospital mortality (all P > 0.05). CONCLUSIONS: The echocardiographic signs of PPM are basically nonspecific with massive pericardial effusion as the most common sign, although no echocardiographic gender differences or association with in-hospital mortality could be identified.

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

BACKGROUND: Primary malignant cardiac tumors rarely occur in clinical care. Little is known about the impact of a parameter on postoperative survival. METHODS: 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%. RESULTS: 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. CONCLUSION: 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.

Right Anterolateral Minithoracotomy versus Median Sternotomy Approach for Resection of Left Atrial Myxoma.

BACKGROUND: Minimally invasive surgery has become the standard approach for several cardiac diseases. In this retrospective study, we compared right anterolateral minithoracotomy (RALT) with standard median sternotomy (SMS) for resection of left atrial myxoma (LAM). MATERIALS AND METHODS: From January 2009 to June 2015, the clinical data of patients who underwent RALT (n = 30) and SMS (n = 36) for resection of LAM in our hospital were collected. The preoperative clinical data and operative results were compared between the two groups. RESULTS: There were no significant differences in aortic cross-clamp and cardiopulmonary bypass time between the two groups. The total incision length was significantly shorter in RALT group compared with SMS group (p < 0.001). For RALT and SMS groups, respectively, the intensive care unit length of stay was 29.2 ± 6.5 versus 43.5 ± 6.9 hours (p < 0.001), and the postoperative hospital length of stay was 5 days (interquartile range [IQR]: 4-6) versus 8 days (IQR: 7-10) (p < 0.001). The total cost in RALT group was 27,000 RMB (IQR: 25,000-29,000) versus 33,000 RMB (IQR: 31,000-35,000) in SMS group (p < 0.001). There were no significant differences in mortality and postoperative complications between the two groups. CONCLUSION: RALT approach for LAM resection can be performed safely with favorable cosmetic outcome, accepted clinical results, and lower cost. It should be considered as a promising alternative to SMS and merit additional study.

Can cardiovascular MRI be used to more definitively characterize cardiac masses initially identified using echocardiography?

In diagnosing cardiac and paracardiac masses, cardiac MRI (CMR) has gained acceptance as the gold standard. CMR has been observed to be superior to echocardiography in characterizing soft-tissue structures and, specifically, in classifying cardiac masses. The aim of our study was to evaluate the association between mortality and cardiac or paracardiac masses initially identified by echocardiography (ECHO) and confirmed by CMR. Between January 2002 and August 2007, a total of 158 patients underwent both ECHO and CMR for the evaluation of cardiac masses that were equivocal or undefined by ECHO. The primary study endpoints were 5-year all-cause mortality and 5-year cardiac mortality. Causes of death as of April 1, 2015 were obtained from medical records or the National Death Index. Patients were analyzed according to mass type determined by CMR using the Kruskal-Wallis test, Kaplan-Meier curves, and the log-rank test. Over a mean duration of follow-up of 10.4 ± 2.9 years (range: 0.01-12 years) post-CMR, the overall all-cause mortality rate was 25.9% (41/158). Median age at death was 76 years and there were 21 females (51.2%). Mortality rates in the different classifications of cardiac masses by CMR were as follows: 20% (1/5) in patients with a Nondiagnostic CMR; 20% (1/5) in Other Diagnoses; 17.9% (7/39) in No Masses (includes Normal Anatomical Variants); 16.7% (3/18) in Benign Masses; 23.8% (15/63) in Fat; 50% (5/10) in Thrombus; and 61.5% (8/13) in Malignant Mass. The mean survival time in patients with No Mass (n = 39) was not significantly longer than patients with any type of cardiac mass (n = 114) (P = .16). No significant difference was found in age at death between patients when grouped by CMR classification (P = .40). However, among CMR-confirmed masses, there were some significant differences by mass classification type (P = .006). During the follow-up period, 26% (41/158) of patients died and 22% (9/41) of the deaths were cardiovascular related; there was no significant difference in mean survival times with respect to cause of mortality (P = .23). In patients with cardiac masses, dually confirmed by ECHO and CMR, significant differences in survival time were observed based upon CMR classified type of mass while CMR was instrumental in obviating invasive biopsy.

Recovering missing mesothelioma deaths in death certificates using hospital records.

BACKGROUND: In Brazil, underreporting of mesothelioma and cancer of the pleura (MCP) is suspected to be high. Records from death certificates (SIM) and hospital registers (SIH-SUS) can be combined to recover missing data but only anonymous databases are available. This study shows how common data can be used for linkage and as an assessment of accuracy. METHODS: Mesothelioma (all sites, ICD-10 codes C45.0-C45.9) and cancer of the pleura (C38.4) were retrieved from both information systems and combined using a linkage algorithm. Accuracy was examined with non-anonymous databases, limited to the state of São Paulo. RESULTS: We found 775 cases in death certificates and 283 in hospital registers. The linkage matched 57 cases, all accurately paired. Three cases, 0.4% in SIM and 1.3% in SIH-SUS, could not be matched because of data inconsistencies. CONCLUSIONS: A computer linkage can recover MCP cases from hospital records not found in death certificates in Brazil.

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

BACKGROUND The 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 AND METHODS A 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. RESULTS Mean 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. CONCLUSIONS Surgical treatment of cardiac tumors is a safe and highly effective strategy associated with good early and long-term outcomes.

Characteristics and Survival of Malignant Cardiac Tumors: A 40-Year Analysis of >500 Patients.

BACKGROUND: The aim of this study was to investigate the incidence, histopathology, demographics, and survival associated with primary malignant cardiac tumors (PMCTs). METHODS AND RESULTS: We queried the Surveillance, Epidemiology and End Results (SEER) 18 registry from the National Cancer Institute for all PMCTs diagnosed from 1973 to 2011. We describe PMCT histopathology and incidence, comparing characteristics and survival of these patients with those of patients with extracardiac malignancies of similar histopathology. From a total of 7 384 580 cases of cancer registered in SEER, we identified 551 PMCTs (0.008%). The incidence of PMCT diagnosis is 34 cases per 100 million persons and has increased over time (25.1 in 1973-1989, 30.2 in 1990-1999, and 46.6 in 2000-2011). Most patients are female (54.1%) and white (78.6%) with median age at diagnosis of 50 years. The most common PMCTs are sarcomas (n=357, 64.8%), followed by lymphomas (n=150, 27%) and mesotheliomas (n=44, 8%). Most patients are diagnosed with tissue sample (96.8%). Although use of chemotherapy is not documented in SEER, 19% of patients received radiation and 44% had surgery. After a median follow-up of 80 months, 413 patients had died. The 1-, 3-, and 5-year survival rates were 46%, 22%, and 17% and have improved over the eras, with 1-, 3-, and 5-year survival rates of 32%, 17%, and 14% for 1973 to 1989 and 50%, 24%, and 19% for 2000 to 2011 (P=0.009). Cardiac sarcomas and mesotheliomas are the most lethal PMCTs, with 1-, 3-, 5-year survival rates of 47%, 16%, and 11% and of 51%, 26%, and 23% compared with 59%, 41%, and 34% for lymphomas, respectively (log rank test P<0.001). Patients with cardiac lymphomas and sarcomas are younger and have worse survival than patients with extracardiac disease of similar histopathology (P<0.001). CONCLUSIONS: PMCTs are extremely rare and continue to be associated with poor prognosis. Over the past 5 decades, the incidence and survival of patients diagnosed with PMCT appear to have increased. Compared with those with extracardiac cancers of similar histopathology, patients with PMCTs are often younger and have worse survival.

Cardiac non-Hodgkin's lymphoma: clinical characteristics and trends in survival.

OBJECTIVES: The purpose of this study was to describe the clinical characteristics and outcomes in cardiac non-Hodgkin's lymphoma (NHL). METHODS: A retrospective analysis of 94 cases of NHL with biopsy-proven cardiac involvement in PubMed between 1990 and 2015. RESULTS: Among cases with cardiac involvement, diffuse large B-cell lymphoma was the most common histologic subtype (58%), followed by T-cell lymphoma (16%), Burkitt's lymphoma (9%), and small lymphocytic lymphoma (6%). Symptomatic heart failure was the most common clinical presentation (34%), and 20% of patients had no cardiac symptoms. Median survival was 3 months (range, 0-72) among all patients. Patients who presented with heart failure had inferior outcomes. Patients with primary, vs. secondary, cardiac involvement had a trend toward superior outcomes. Importantly, chemotherapy treatment was associated with a prolongation in median survival (18 vs. 1 month, HR 0.16, 95% CI, 0.47-0.54, P = 0.0003), and patients diagnosed in the chemo-immunotherapy era demonstrated a trend toward better outcomes. Median survival was not reached among patients with B-cell malignancies who were alive for 1 month after the diagnosis. CONCLUSION: Pathologic lymphomatous involvement of cardiac tissue should be considered in the evaluation of patients with NHL. Durable remissions can be achieved in B-cell NHL with cardiac involvement, and thus, therapy should be considered in such cases.