Malignant Cardiac Tumors Research Articles

Successful Management of Primary Cardiac Lymphoma with Minimal Debulking Surgery Combined with Adjuvant Chemotherapy

Primary cardiac lymphoma (PCL) is a rare cardiac tumor with poor prognosis. Palliative chemotherapy is still considered to be the standard management tool for PCL. A case of a 58-year-old man with a large right-heart tumor is presented. Echocardiography showed no abnormal findings except mild tricuspid stenosis with dynamic obstruction. To prevent sudden right heart failure, we pursued on-pump beating resection. After resection of the tumor near the tricuspid valve and confirmation of normalized hemodynamics by intraoperative transesophageal echocardiography, we decided not to perform a further debulking procedure, such as resection or reconstruction of the atrial/ventricular wall. The postoperative course was uneventful and the patient tolerated six cycles of adjuvant chemotherapy well. Currently, the patient visits the outpatient clinic regularly without definite evidence of lymphoma involvement on follow-up imaging studies. In cases where a rare malignant cardiac tumor is suspected, surgical resection should be considered a diagnostic tool for tissue confirmation, a therapeutic tool for hemodynamic correction, and a preventive strategy for sudden cardiac death. Additionally, a minimal debulking procedure focusing on the area of hemodynamic disturbance appears to be sufficient in PCL cases.

Circulation : Clinical Summaries

<i>Circulation</i> : Clinical Summaries

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

The aim of this study was to investigate the incidence, histopathology, demographics, and survival associated with primary malignant cardiac tumors (PMCTs). 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). 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.

Pericardial angiosarcoma: an interesting case report

Angiosarcoma, despite being the most common primary malignant tumour of the heart and pericardium, is itself an extremely rare tumour with a 0.001 to 0.003 % prevalence in an autopsy series. Angiosarcoma is the most common form of malignant cardiac tumour, having a tendency to occur in the third to fifth decade of life, with a distinct male preponderance. The tumour is often silent and symptomless for a long period of time, presenting late with locoregional metastasis which hampers the operability and prognosis. Metastasis is mainly to the mediastinal lymph nodes, lungs and vertebra. We present the case of a 58-year-old normotensive diabetic male who presented with dyspnea on exertion and recurrent pericardial effusion with an impending cardiac tamponade. It was identified as a large pericardial mass on CT thorax, and partial pericardiectomy was done. Histopathology identified it as a poorly differentiated carcinoma. It was only by immunohistochemistry that the diagnosis of pericardial angiosarcoma was confirmed.

Imaging findings in cardiac masses (Part i): Study protocol and benign tumors

Cardiac masses represent a diagnostic challenge because decisions about treatment are based on imaging techniques. Echocardiography, magnetic resonance (MR) and computed tomography (CT) are fundamental for the detection, characterization, and staging of cardiac masses as well as for planning their treatment. Most primary cardiac tumors are benign; myxomas, papillary fibroelastomas, and lipomas are the most common. The location of the tumors and its characteristics on CT and MR orient the etiologic diagnosis in most cases.This article describes the protocols for CT and MR studies of cardiac masses as well as the morphologic findings, predominant locations, and most useful characteristics for characterizing benign cardiac masses and establishing the differential diagnosis with malignant cardiac tumors and non-neoplastic pseudotumors.

Echocardiographic Features of Cardiac Angiosarcomas: The Mayo Clinic Experience (1976-2013).

Cardiac angiosarcoma is the most common primary malignant cardiac tumor. The dismal prognosis and nonspecific symptomatology underscore the need for an accurate and cost-effective approach to the identification and characterization of this rare tumor. Mayo Clinic tissue registry archives were queried for all histologically confirmed cases of cardiac angiosarcoma (1976-2013) with available imaging data. Echocardiograms were retrospectively reviewed. Thirty-three cases of cardiac angiosarcoma were identified; of these, 17 had echocardiograms available (mean age, 46 years; six men). Transthoracic echocardiography (TTE) as the initial diagnostic test had 75% sensitivity for visualizing primary cardiac angiosarcoma (9/12 patients). Tumor extension into the pericardium was common and pericardial effusion was present in 15 patients (88%); however, pericardial fluid cytology was negative for malignancy in all tested patients (n = 15). Left ventricular ejection fraction (LVEF) was preserved in 16 patients (94%) (average LVEF, 62%). Right ventricular function was mildly reduced in two patients (12%) at initial presentation. Tricuspid valve obstruction was present in three patients (18%; mean diastolic gradient, 6.3 mmHg [range, 3-11 mmHg]). The sensitivity of TTE as the first diagnostic imaging modality compared favorably with computed tomography. Pericardial effusion was common, but pericardial fluid cytology was negative in all patients who underwent pericardiocentesis. The absence of a stalk was a universal finding that may help distinguish angiosarcoma from benign, primarily pedunculated tumors such as myxoma and papillary fibroelastoma.

Hallazgos de imagen de las masas cardíacas (parte I): protocolo de estudio y tumores benignos

Cardiac masses represent a diagnostic challenge because decisions about treatment are based on imaging techniques. Echocardiography, magnetic resonance (MR) and computed tomography (CT) are fundamental for the detection, characterization, and staging of cardiac masses as well as for planning their treatment. Most primary cardiac tumors are benign; myxomas, papillary fibroelastomas, and lipomas are the most common. The location of the tumors and its characteristics on CT and MR orient the etiologic diagnosis in most cases.This article describes the protocols for CT and MR studies of cardiac masses as well as the morphologic findings, predominant locations, and most useful characteristics for characterizing benign cardiac masses and establishing the differential diagnosis with malignant cardiac tumors and non-neoplastic pseudotumors.

Malignant cardiac tumors: diagnosis and treatment.

Primary malignant cardiac tumors are represented by sarcomas and non-Hodgkin lymphomas. They are rare, affect mostly patients in the fourth decade of life and have a severe prognosis. Both the diagnosis and the treatment require a multidisciplinary approach, and the cardiologist plays a central role both in the diagnosis and in the follow-up. The prognosis may be improved by a careful planning of surgery and by the use of multimodality treatment, including complementary chemotherapy and radiation therapy. A strict follow-up must be planned even after apparently complete cure.

The current trends of mortality following congenital heart surgery: the Japan Congenital Cardiovascular Surgery Database.

Whereas surgical outcomes of congenital heart surgery have improved during the past two decades, there are still measurable postoperative mortalities in this field. This study is aimed at evaluating the current situation of mortality following congenital heart surgery. Data on all registered 28 810 patients in The Japan Congenital Cardiovascular Surgery Database (JCCVSD) between 2008 and 2012 were analysed, except for patients with degenerative cardiomyopathy including dilated, restrictive and hypertrophic cardiomyopathy, and pathologically or histologically malignant cardiac tumours. The number of registered cases increased every year, and reached ∼9000 cases in 2012. The median age at surgery was 0.8 years (range, 0-82). More than half of the patients (54%) who underwent surgery were <1 year old, and 6.0% of all patients were over 18 years old (adults). In this study, all mortalities within 90 days after the operation and mortality at discharge beyond 90 days of hospitalization were defined as '90-day and in-hospital mortality'. The 30-, 90-day and in-hospital mortality rates were 2.3, 3.5 and 4.5%, respectively. The mean and median durations from surgery to death were 61 ± 89 and 28 days (range, 0-717), respectively. Whereas 658 mortalities (51%) occurred within 30 days of surgery, 265 (21%) occurred later than 90 days after surgery. A total of 3630 patients (13%) were hospitalized for more than 90 days after the operation; of those, 3365 patients survived at discharge (93%). Cardiac problems were the most frequent causes of death after the surgery at any point in time, and 7.1 per 1000 patients died at over 30 days after the operation due to solely cardiac. The investigation of JCCVSD revealed that about a half of mortalities occurred later than 30 days; hence 90-day and in-hospital mortality would be a good discriminator that accurately represented the current situation of mortality after congenital heart surgery. Mortalities long after the operation due to post-cardiotomy heart failure without any other lethal complications were still not rare.

Diagnostic value of 18F-FDG PET/CT in differentiating the cardiac benign from malignant lesions

Objective To explore the clinical value of 18F-FDG PET/CT in differentiating between cardiac benign and malignant lesions. Methods From September 2009 to September 2012, a total of 9 (7 males, 2 females, average age (46±22) years) patients with space-occupying lesions of the heart underwent whole body 18F-FDG PET/CT. 18F-FDG PET/CT diagnosis was made according to morphologic features and SUVmax of lesions. The final diagnoses were confirmed by either histopathology or clinical data and follow-up. Two-sample t test was used for data analysis. Results Among 9 cases of cardiac occupying lesions, 4 cases were malignant tumors, 4 cases were benign tumors (including 2 lipomas, 1 myxoma, and 1 fibroma), 1 case was thrombus. The average SUVmax of malignant tumors was 18.99±14.58, which was significantly higher than that of benign tumors (2.35±1.46, t=-2.58, P<0.05). With a cut-off SUVmax of 4.6, 18F-FDG PET/CT could be used to noninvasively detect malignant tumors with a sensitivity of 4/4, specificity of 4/5, and accuracy of 8/9. Only 1 thrombus was misdiagnosed. Conclusion 18F-FDG PET/CT can aid the noninvasive differential diagnosis of cardiac malignant tumors from benign lesions. Key words: Heart neoplasms; Tomography, emission-computed; Tomography, X-ray computed; Deoxyglucose

Primary malignant tumors of the heart: Outcomes of the surgical treatment.

Malignant cardiac tumors are rare and have an extremely poor prognosis even when complete resection is attempted. The aim of this study was to review the experience of primary malignant cardiac tumors in 2 Italian academic hospitals. The hospital records were searched to identify patients with primary malignant cardiac tumors who underwent surgery between January 1979 and December 2012. Secondary cardiac tumors, whether metastatic or invasive, were excluded as were primary sarcomas of the great arteries. Fourteen patients selected from our institution's surgical series were identified. Eleven (78.6%) were men and 3 (21.4%) were women, and the mean age at surgery was 47.4 years. The most common histological type was angiosarcoma (28.6%). The mean survival was 28.8 ± 28 months and it was better in men than in women (30.5 ± 8.7 vs. 21.1 ± 3.2 months). Patients with a radical resection at the first surgery had a longer survival compared to patients with a partial resection (39.9 ± 23.2 vs. 24 ± 4 months). The treatment outcome for patients affected by primary malignant heart tumors remains poor. Aggressive surgery alone does not provide good results in terms of survival rate. A new multidisciplinary approach is mandatory to improve long-term survival.

Severe Right Atrial Compression by a Rapidly Growing Cardiac Mass

A 42-year-old man without a past cardiovascular history arrived in the emergency department reporting progressive chest pain, abdominal discomfort, dyspnea at rest, and orthopnea of 4 days duration. An abdominal ultrasound revealed significant pericardial and bilateral pleural effusion, with hepatomegaly and ascites of a probable congestive nature. A transthoracic echocardiogram showed severe pericardial effusion with data of hemodynamic compromise, and a right retroauricular mass anchored to the posterior atrial wall (Figures 1A and 2; Movies I and II in the online-only Data Supplement). Figure 1. A , Apical 4-chamber view transthoracic echocardiogram showing concentric severe pericardial effusion (PE), right (RV) and left (LV) ventricle, and tumor (arrow). B , CT scan showing tumor mass (arrow) attached to the right atrium (RA). C , Apical 4-chamber view with color flow Doppler demonstrating in the right chambers flow acceleration (arrow) secondary to extrinsic tumor compression. D , CT scan …

Cardiac masses due to tumors (diagnosis, clinical manifestations and surgical treatment)

Aim. To summarize the experience on the diagnosis and surgical treatment of primary benign and malignant cardiac tumors. Methods. The paper describes 37 clinical observations of endocavitary primary benign and malignant cardiac tumors: 26 (70.3%) cases of cardiac myxoma, 5 (13.5%) - rhabdomyosarcoma, 2 (5.4%) - angiosarcoma, 1 (2.7% ) - leiomyosarcoma, 1 (2.7%) - fibrosarcoma, 1 (2.7%) - liposarcoma. Patients were 15 (40.5%) males and 22 (59.5%) females aged 18 to 65 years. Endocavitary cardiac tumors were diagnosed by noninvasive tests: echocardiography, computed tomography, magnetic resonance imaging. Results. Patients with primary benign and malignant cardiac tumors had variable clinical manifestations. Symptoms of astenoneurotic syndrome, auscultatory and cardiophonographic signs simulating acquired or congenital valvular heart diseases, which often have a positional relationship; low-grade fever of unknown origin, weight loss were registered. Paraneoplastic syndrome was characterized by an increase in erythrocyte sedimentation rate, leukocytosis, monocytosis, dysproteinemia, polycythemia, hypochromic anemia, increased levels of C-reactive protein. Surgical excision of 25 cardiac myxomas and 8 malignant tumors of the heart was performed with cardiopulmonary bypass, pharmacological cardioplegia and general hypothermia. In 1 case the tumor resection was combined with coronary artery bypass grafting. In 3 patients suffering from malignant tumors, explorative thoracotomy was performed. Surgical revision, emergency and planned microscopic studies of removed cardiac tumors allowed to establish the final clinical diagnosis. Hospital mortality among patients operated for cardiac myxomas was 4.0%, for cardiac malignant tumors - 27.3%. Conclusion. Timely surgical treatment of patients with primary cardiac benign tumors (myxomas) leads to recovery and is accompanied by a relatively low mortality; better results of patients with cardiac endocavitary tumors treatment depend on the early detection and timely radical surgical excision.

MRI assessment of cardiac tumours: part 2, spectrum of appearances of histologically malignant lesions and tumour mimics.

Cardiac magnetic resonance imaging (MRI) is the reference standard technique for assessment and characterization of a suspected cardiac tumour. It provides an unrestricted field of view, high temporal resolution and non-invasive tissue characterization based on multi-parametric assessment of the chemical micro-environment. Sarcomas account for around 95% of all primary malignant cardiac tumours with lymphoma, and primary pericardial mesothelioma making up most of the remainder of cases. By contrast cardiac metastases are much more common. In this article we review the MRI features of the spectrum of histologically malignant cardiac and pericardial tumours as well as some potential tumour mimics.

Value of CMR for the Differential Diagnosis of Cardiac Masses

ObjectivesThe goal of this study was to evaluate the diagnostic value of CMR features for the differential diagnosis of cardiac masses. BackgroundDifferentiation of cardiac tumors and thrombi and differentiation of benign from malignant cardiac neoplasms is often challenging but important in clinical practice. Studies assessing the value of cardiac magnetic resonance (CMR) in this regard are scarce. MethodsWe reviewed the CMR scans of patients with a definite cardiac thrombus or tumor. Mass characteristics on cine, T1-weighted turbo spin echo (T1w-TSE) and T2-weighted turbo spin echo (T2w-TSE), contrast first-pass perfusion (FPP), post-contrast inversion time (TI) scout, and late gadolinium enhancement (LGE) sequences were analyzed. ResultsThere were 84 thrombi, 17 benign tumors, and 25 malignant tumors in 116 patients. Morphologically, thrombi were smaller (median area 1.6 vs. 8.5 cm2; p < 0.0001), more homogeneous (99% vs. 46%; p < 0.0001), and less mobile (13% vs. 33%; p = 0.007) than tumors. Hyperintensity compared with normal myocardium on T2w-TSE, FPP, and LGE were more common in tumors than in thrombi (85% vs. 42%, 70% vs. 4%, and 71% vs. 5%, respectively; all p < 0.0001). A pattern of hyperintensity/isointensity (compared with normal myocardium) with short TI and hypointensity with long TI was very frequent in thrombi (94%), rare in tumors (2%), and had the highest accuracy (95%) for the differentiation of both entities. Regarding the characterization of neoplastic masses, malignant tumors were larger (median area 11.9 vs. 6.3 cm2; p = 0.006) and more frequently exhibited FPP (84% vs. 47%; p = 0.03) and LGE (92% vs. 41%; p = 0.001). The ability of CMR features to distinguish benign from malignant neoplasms was moderate, with LGE showing the highest accuracy (79%). ConclusionsCMR features demonstrated excellent accuracy for the differentiation of cardiac thrombi from tumors and can be helpful for the distinction of benign versus malignant neoplasms.

Pediatric cardiac tumors: clinical and imaging features.

Cardiac tumors in children are rare, are more commonly benign, and differ in types when compared with those in adults. Rhabdomyoma, teratoma, and fibroma are the most common cardiac tumors in fetuses and neonates. In infants and children, the most common cardiac tumors are rhabdomyoma and fibroma, which are benign primary cardiac tumors, whereas in adults, thrombus is the most common cardiac mass and myxoma is the most common primary cardiac tumor. Sarcomas are the most common primary malignant cardiac tumors in both children and adults. Metastatic cardiac tumors are less common in children than in adults and include sarcoma, lymphoma, testicular cancer, and Wilms tumor. Patients with cardiac tumors can be asymptomatic or may present with murmur, arrhythmia, heart failure, or sudden death. Echocardiography is the primary modality for initial evaluation of symptomatic patients. Cardiac magnetic resonance imaging provides further characterization of cardiac masses and tumors for diagnostic purposes and can be used to assess cardiac and extracardiac involvement for treatment planning. Treatment varies from conservative management or surgical resection to transplantation, depending on the diagnosis and the patient's symptoms. Rhabdomyoma typically regresses spontaneously during early childhood and does not require surgical intervention if asymptomatic. However, fibroma is generally resected because it does not regress. An understanding of the types of cardiac tumors that occur in infants and children, their clinical implications and associations, and their imaging appearances will facilitate patient management.

Multimodality Imaging of a Cardiac Angiosarcoma

Introduction: While primary malignant tumors of the heart are rare, angiosarcomas are the most common cardiac malignant tumors. Case Presentation: We describe a 23-year-old woman who presented with a right atrial mass, which was discovered to be a cardiac angiosarcoma. We demonstrate the use of several noninvasive imaging modalities along with pathology confirmation for the definitive and comprehensive diagnosis of a cardiac angiosarcoma, a rare entity by itself. Conclusions: With the increasing availability of noninvasive imaging techniques, the diagnosis of angiosarcomas can be made at earlier stages. If angiosarcomas are left untreated, their prognosis is very poor. Therapeutic options include surgical excision, chemotherapy, radiation therapy, and heart transplantation or a combination of these.

Atrial fibrillation as the first clinical presentation of an adenoid cystic bronchial carcinoma.

A previously healthy 50-year-old man was admitted with a repeated rapid heartbeat. Electrocardiograph on admission detected atrial fibrillation with an irregular ventricular rate of 170 beats/min for the first time. Transthoracic and transoesophageal echocardiography showed a 5 × 3.5 cm large sessile mass attached to the lateral wall of the left atrium (Fig. 1a) and a mild pericardial effusion. Computed tomography scan of the chest showed a lobulated lymphoma-like infracarinal mass 7.5 × 5 × 5 cm in size compromising the oesophagus and the left atrium, further enlarged hilar lymph nodes in the pathway of draining lymph ducts and bilateral pleural effusion (Fig. 1b). Cranial and abdominal CT was normal. An endobronchial ultrasound biopsy of the enlarged lymph nodes was performed. Histological and immunocytochemical examination confirmed an adenoid cystic bronchial carcinoma (Fig. 1c, d). The patient was immediately transferred to an oncology centre for further treatment. Fig. 1 Transoesophageal echocardiogram (a) showing a left atrial mass (arrows). CT scan of the chest shows a polycyclic infracarinal tumour (arrows) with enlarged hilar lymph nodes and bilateral pleural effusion (b). An adenoid cystic bronchial carcinoma characteristically ... Secondary cardiac neoplasms with an incidence of up to 1 % at autopsy are more than 20 times more common than primary cardiac tumours [1, 2]. The most common secondary malignant cardiac tumours including both metastasis and local extension were bronchial carcinoma, oesophageal carcinoma, carcinoma of the breast and lymphoma [3, 4]. Certain patients with cardiac neoplasms manifest only recurrent supraventricular or ventricular arrhythmias, most likely due to the irritative effect of tumour invading cardiac muscle [5]. Rarely, cardiac involvement may be the first clinical feature of malignancy. We present a case of an intrathoracic malignancy encroaching upon the heart and causing atrial fibrillation as its first presenting symptom.

Primary malignant cardiac tumors

The literature on primary malignant cardiac tumors is relatively limited because of their rare incidence. This study aimed to provide a proposed treatment strategy for primary malignant cardiac tumors. The follow-up outcomes of 29 patients with primary malignant cardiac tumors operated, and 8 primary malignant cardiac tumors considered not operable from 1985 to 2013 in the First Affiliated Hospital of China Medical University were retrospectively analyzed. Of operation receivers, ten patients had positive surgical margins and nineteen patients had negative surgical margins. Eleven patients received a post-operative neoadjuvant chemotherapy. Patients rejected to surgery had a lower survival compared with operation receivers (15 vs 23 months, P = 0.011). However, there were no significant differences in survival in patients rejected to surgery than in patients who had positive surgical margins (15 vs 16 months, P = 0.874). Patients who had positive surgical margins had a median overall survival duration of only 16 months, whereas patients with negative surgical margins had a median overall survival duration of 27 months (P = 0.002). There were no significant differences in survival in patients with receiving a post-operative adjuvant chemotherapy than in the rest of the population (20 vs 25 months, P = 0.150). The prognosis for patients with primary malignant cardiac tumors remains very poor. Each patient should be managed on an individual basis, and variety of treatment strategy should be performed. Maximizing the possibility of obtaining negative surgical margins may prolong survival.

Primary right atrial angiosarcoma misdiagnosed as aortic intramural hematoma

The overall incidence of cardiac tumor is very low, and malignant cardiac tumors account for only 25% of all cardiac tumors. Angiosarcomas are the most common type of malignant cardiac tumors, characterized by rapidly proliferating, extensively infiltrating anaplastic cells derived from blood vessels and lining irregular blood-filled spaces. We present a 26-year-old man with angiosarcoma involving the right atrium, which was misdiagnosed as aortic intramural hematoma by computed tomography, finally confirmed by transesophageal echocardiography during the operation.