Primary Malignant Cardiac Tumors Research Articles

Beating the Odds – 4-year Survival with Primary Cardiac Angiosarcoma

A 49-year-old man presented with a 6-week history of exertional dyspnoea. Echocardiogram demonstrated a pericardial effusion. Pericardiocentesis was performed, but fluid cytology was negative for malignancy. Nine months later, repeat echocardiogram showed a right atrial mass and cardiac MRI showed a heterogeneous mass suspicious for cardiac sarcoma. Histology confirmed a cardiac angiosarcoma, deemed inoperable due to cardiac wall infiltration. He commenced weekly paclitaxel treatment and demonstrated a 70% reduction in cardiac mass on CT after two cycles of therapy. An application for cardiac transplant was refused. His tumour showed no response to two further cycles of paclitaxel and so he was switched to liposomal doxorubicin. This was stopped prematurely due to grade 3 hand-foot skin toxicity. CT scans also demonstrated further disease progression. He was transferred to the Royal Marsden for resection of his primary tumour, with clear margins on resection specimen. He remained disease-free for ten months when liver lesions were identified on staging CT. Biopsy confirmed metastatic disease. He was treated with a tyrosine kinase inhibitor and an mTOR inhibitor but ultimately progressed and therapy was discontinued at patient request. Four months later he represented with symptomatic anaemia. CT showed disease progression with a large hepatic metastasis eroding into omentum. He was treated with further chemotherapy but passed away 51 months after diagnosis. We discuss the treatment options available for this rare condition. Primary malignant cardiac tumours are rare. Clinical diagnosis is often delayed so patients present with advanced stage disease, usually involving the right atrium, often inoperable at presentation. Cardiac angiosarcoma is particularly aggressive, with median survival of 6-12 months. The benefit of chemotherapy in this setting is usually short-lived; with the regimens most commonly used involving doxorubicin or paclitaxel, although more recent studies have suggested a role for targeted agents such as tyrosine kinase inhibitors or mTOR inhibitors. To our knowledge, this case represents the longest documented survival in adult cardiac angiosarcoma and highlights the need for multidisciplinary care in this rare disease.

Cardiac Relapse of Acute Myeloid Leukemia after Allogeneic Hematopoietic Stem Cell Transplantation

Secondary or metastatic cardiac tumors are much more common than primary benign or malignant cardiac tumors. Any tumor can cause myocardial or pericardial metastasis, although isolated or combined tumor invasion of the pericardium is more common. Types of neoplasia with the highest rates of cardiac or pericardial involvement are melanoma, lung cancer, and breast and mediastinal carcinomas. Acute myeloid leukemia (AML) is the most common type of acute leukemia in adults. Initial treatment involves chemotherapy followed by consolidation treatment to reduce the risk of relapse. In high-risk patients, the treatment of choice for consolidation is hematopoietic stem cell transplantation (HSCT). Relapse of AML is the most common cause of HSCT failure. Extramedullary relapse is rare. The organs most frequently affected, called “sanctuaries,” are the testes, ovaries, and central nervous system. We present a case with extramedullary relapse in the form of a solid cardiac mass.

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.

Circulation : Clinical Summaries

<i>Circulation</i> : Clinical Summaries

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.

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.

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.

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.

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.

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.

Diagnosis and surgical treatment of primary malignant cardiac tumors

Diagnosis and surgical treatment of primary malignant cardiac tumors

Primary Cardiac Tumours: Our Experience

Introduction Primary cardiac tumours are rare with autopsy incidence of less than 0.1 percent. We present our experience on surgical treatment of such tumours. Methods Since 2001, fifteen patients underwent surgical intervention for primary cardiac tumour at our centre. Mean age was 46.5 ± 17.5 years (range 20 to 73 years). There were eight female patients. Thirteen patients had atrial myxoma and the remaining two had primary malignant tumours. Surgical excision of the tumour was done under cardiopulmonary bypass and cardioplegic cardiac arrest. Results Complete excision was possible in all benign cardiac tumours where as excision was possible in only one of the two malignant tumours. One patient with suspected angiosarcoma had biopsy taken from the heart. There was no operative mortality. All the patients with atrial myxoma were symptom free and free of recurrence on follow-up echocardiography. The patient with suspected angiosarcoma (histopathology was reported as hemangioma only) presented with disseminated disease and metastasis to thoracic spine with paraplegia three months postoperatively. The other patient with rhabdomyosarcoma who had complete excision of tumour followed by repair of the defect is currently on adjuvant chemotherapy. Conclusion Surgical excision of primary benign cardiac tumours is possible with excellent surgical outcome where as outcome is guarded in primary malignant cardiac tumours. DOI: http://dx.doi.org/10.3126/njh.v8i1.8329 Nepalese Heart Journal Vol.8(1) 2011 pp.8-11

Recurrent primary cardiac osteosarcoma: a case report and literature review

Primary malignant cardiac tumors are very rare. Among malignant tumors, sarcomas occupy first place. In particular, primary cardiac osteosarcoma is extremely rare. To the best of our knowledge, only 42 cases have been reported worldwide. Cardiac malignant tumors usually require complex operations due to the difficulty in completely removing the tumor with acceptable free surgical margins and because of the proximity to vital structures. The current multimodality treatment strategies for cardiac sarcoma are still suboptimal, and surgery in particular frequently has unsatisfactory results. We report a case of recurrent primary cardiac osteosarcoma in a young male who underwent trans-sternal right pneumonectomy and a wide resection of the left and right atrium followed by reconstruction with heterologous pericardium under extracorporeal circulation. The patient died 6months after the operation due to local and systemic disease recurrence.

Primary pediatric cardiac malignancies: the SEER experience

Pediatric cardiac malignancies are exceedingly rare. We sought to examine demographics, presentation, and outcomes for this pathology. The SEER registry from 1973 to 2008 was queried for all patients <20 years of age with cardiac malignancies. A total of 25 pediatric patients were identified with primary cardiac malignancies, with age-adjusted incidence of 0.00686 per 100,000 United States population. Median age at diagnosis was 10 years. The majority of patients were adolescent (n = 13, 52 %), Caucasian (n = 17, 68 %) and males (n = 14, 56 %). The most common histology was soft tissue sarcoma (n = 10, 40%), followed by non-Hodgkin lymphoma and teratoma (both n = 3, 12 %). Six patients presented with distant disease. More than half of patients (n = 16, 64 %) underwent surgical resection, while four patients (16 %) underwent radiation. The mean survival time for the cohort was 47 ± 67 months, with 14 (56 %) patients dying over the study period. Lymphomas had significantly longer survival than other malignancies (108 ± 66 vs. 36 ± 66, p = 0.03), while lack of surgical treatment was associated with worse survival (p = 0.016). Primary malignant cardiac tumors are exceedingly rare in pediatric patients. They are most commonly soft tissue sarcomas and lymphomas demonstrated longer survival.

Recurrence of Primary Cardiac Rhabdomyosarcoma without Methastasis Two Years After Surgery

Primary malignant cardiac tumors are rare. Rhabdomyosarcoma is the most common of these lesions. These tumors usually arise from the ventricular walls. They sometimes arise from the atrial walls and mimic atrioventricular valve stenosis. A case of recurrence of primary cardiac rhabdomyosarcoma without metastasis to other organs in a 34 year old woman is presented. The tumor arose from the posterior wall of the left atrium and extended to the posterior mitral valve leaflet. Histopathology confirmed re currence of the cardiac rhabdomyosarcoma. Although cardiac rhabdomyosarcomas are highly lethal, operation indicated. Clarify diagnosis, relieve symptoms and improve survive intracardiac mass must be excision as as extensively as possible in these cases in order to prevent recurrence of tumor.

Primary and metastatic cardiac tumors: imaging characteristics, surgical treatment, and histopathological spectrum: a 10-year-experience at a German heart center

Tumors of the heart are rare compared to other cardiac diseases. Their clinical symptoms vary from absent to nonspecific. This great variation and general paucity of symptoms related to cardiac tumors often result in delayed diagnosis and treatment. We retrospectively evaluated all patients who underwent cardiac surgery for a space-occupying lesion in the observation period between 2000 and 2010 at our hospital. Clinicopathological features, imaging characteristics, and disease outcomes were analyzed, and the results were compared with the available English literature. During the last 10 years, 84 patients underwent resection of a cardiac mass at our center, i.e., 0.85% of the total number (n=9829) of all cardiac surgical operations performed in that period. The part of primary cardiac tumors was 73.8% (n=62; 59 benign and 3 malign tumors). In nine cases (10.7%), secondary cardiac tumors represented metastases of malignant tumors from different extracardiac locations. In 13 cases (15.5%), the mass represented cardiac thrombus. The majority of cardiac tumors were benign, and most of them were cardiac myxomas (n=48). Papillary fibroelastoma was the second most common primary tumor. Our data in this study were comparable to the literature regarding the frequency and allocation of the different cardiac tumors. Our data are in line with previous reports that patients with benign cardiac tumors profit from surgical resection compared to those subjected to conservative treatment with the risk of central or peripheral embolisms. Concerning malignant primary cardiac tumors and cardiac metastases, surgery represents only a palliative strategy in most of the cases.

Cardiac Angiosarcoma Presenting with Tamponade

Primary cardiac angiosarcoma is extremely rare, but it is the most common primary malignant cardiac tumor. We herein present the case of a 51-year-old man who presented with symptoms of acute right heart failure, secondary to pericardial tamponade. Pericardiocentesis showed bloody fluid with negative pathology. Repeat 2-D echocardiography and a trans-esophageal echocardiogram showed a right atrial mass. The patient underwent surgery and adjuvant chemotherapy, but died seven months after the diagnosis. Despite being rare, cardiac angiosarcoma should be included in the differential diagnosis of bloody pericardial effusions, even with negative early investigations. The prognosis of the disease is usually poor. Treatment is mainly surgical resection if the cancer is localized, and can include neadjuvant and adjuvant chemotherapy, radiation treatment, and immunotherapy.

Leiomyosarcoma with partial rhabdomyoblastic differentiation: First case report of primary cardiac origin

BackgroundLeiomyosarcoma occurring as a primary cardiac tumor has been known as an extremely rare condition. Previous studies of leiomyosarcoma with rhabdomyoblastic differentiation have conducted to those arisen from another site, and they indicated a poorer prognosis of this tumor.Case presentationA 69-year-old woman was referred to our hospital for an operation concerning umbilical hernia. Subsequent imaging examinations before an operation indicated the presence of primary cardiac malignant tumor due to its atypical shape. And then, it was surgically removed. Histopathologically, tumor cells consisted of two different types: spindle and polyhedral cells. Immunohistochemically, it is interesting to note that 2.1% of spindle cells and 23.1% of polyhedral cells showed positive reactivity for myogenin. Furthermore, we performed double-immunostaining for alpha-smooth muscle actin (SMA) and myogenin. The rates of alpha-SMA and myogenin double negative, alpha-SMA single positive, myogenin single positive, and alpha-SMA and myogenin double positive in spindle cells were estimated as 69.1%, 28.8%, 1.1% and 1.0%, respectively. In contrast, the rates in polyhedral cells were estimated as 76.9%, 0.0%, 23.1%, and 0.0%, respectively.ConclusionOur immunohistochemical evaluation suggested that rhabdomyoblastic differentiation in leiomyosarcoma might be generated not only by de novo generation from mesenchymal cells. To the best of our knowledge, this is the first case of primary cardiac leiomyosarcoma with partial rhabdomyoblastic differentiation.

Non-diagnosed primary malignant cardiac tumor as a cause of sudden death in a 52-year-old man. A case report with medico-legal implications

We describe the case of a malignant fibrous histiocytoma (MFH) of the heart, presenting with an obstinate cough and clinically misdiagnosed as adult respiratory distress syndrome, in a 52-year-old man. He was diagnosed as pulmonary tuberculosis four years ago. Although his tuberculosis was believed to have cured, he often went to hospital for cough and palpitation. Echocardiogram was conducted only once on the patient four years ago but no abnormalities were found. During the day his condition deteriorated. He went to the emergency department, where he was given intravenous drugs against cough and was sent home. On the way back, his condition deteriorated dramatically so that his son drove back to the emergency room, where he collapsed and sustained cardiac arrest; resuscitation efforts were unsuccessful. Autopsy revealed a large tumor located in the left atrium. Histological and immunohistochemical investigations of the mass established the diagnosis of primary cardiac MFH. Sudden cardiac death, caused by the MFH was ascertained as the cause of death. The morphological and pathological findings are presented, the difficulty to diagnose primary cardiac MFH and the medico-legal implications are discussed.