Primary Malignant Cardiac Tumors Research Articles

Primary cardiac synovial sarcoma: A case report and literature review

Primary cardiac synovial sarcoma is a rare disease. A 51-year-old man visited our hospital with the chief complaint of palpitations and shortness of breath while exercising. Copious bloody pericardial effusion and a multicystic intrapericardial tumor were detected. A primary cardiac malignant tumor was suspected, an open-chest tumor resection was performed with the objectives of diagnosis and treatment. Histologically, the tumor cells were uniformly spindle-shaped with an ovoid or oval nucleus, they had proliferated in fascicular fashion. In addition myxoid degeneration, a hemangiopericytomatous vascular pattern and pseudorosette formation were seen in some areas of the tumor. Based on the histopathological and immunohistochemical findings and reverse transcription polymerase chain reaction detection of SS18-SSX1 fusion transcripts, a monophasic fibrous type synovial sarcoma was diagnosed. Postoperative radiation therapy was administered and there had been no recurrence 9 months after the surgery.

Hemorrhagic Tamponade Due to Cardiac Angiosarcoma

Prognosis of angiosarcoma, the most common primary malignant cardiac tumor, is very poor. An early detection and treatment may extend survival beyond one year. Newer imaging modalities, including magnetic resonance imaging (MRI), play an important role in the evaluation of cardiac masses. The case of a man admitted to the emergency room for a cardiac tamponade is reported. Thoracic computed tomography and MRI diagnosed a pericardial tumor, for which surgical biopsy revealed an angiosarcoma. Chemotherapy was started, and the patient survived for 28 months. Etiologies of hemorrhagic tamponades are discussed, as well as treatment of cardiac angiosarcoma.

Right Heart Sarcomas

Primary cardiac tumors are unusual, and primary cardiac sarcomas constitute a rare subset of these. In cardiac sarcoma, unlike many malignancies, the histologic cell type does appear to affect the treatment options or prognosis in a significant way. The presenting symptoms, treatment options and, indeed, prognosis are largely controlled by the tumor's anatomic location. We have proposed a classification system based on anatomic location that divides cardiac sarcoma into left heart, right heart and pulmonary artery sarcomas. In our experience, right heart sarcoma tends to be bulky, grow in a more exophitic manner, be more infiltrative, and metastasize earlier than left heart or pulmonary artery sarcoma. Right heart sarcoma also presents less often in congestive heart failure or with compromised hemodynamic status than left heart and pulmonary artery sarcoma, which are usually highly symptomatic at presentation. The prognosis for right heart sarcoma without surgery is dismal. Complete surgical resection remains the goal of therapy and the only treatment modality shown to increase survival. Complete surgical resection is complicated both by the bulky infiltrative nature of right heart sarcoma and the high incidence of metastatic disease at presentation. The current approach of our cardiac sarcoma group to right heart sarcoma has been to begin neoadjuvant chemotherapy once a definitive tissue diagnosis of sarcoma is achieved. After 4 to 6 rounds of chemotherapy, the patient is considered for surgical resection. This standardized treatment has been approved in our IRB protocol: a clinical trial to assess the safety and Efficacy of a novel radical tumor reSection Procedure used in conjunction with nEoadjuvant chemotheRapy to treat malignant primary right heart cardiac tumOrs - the ESPERO trial. This protocol is designed to compare our existing 24 index cases of surgical resection of right heart sarcoma using a nonstandardized treatment plan, with routine neoadjuvant chemotherapy, and a standardized treatment plan to see if the rate of microscopically complete resection can be improved from its current level of 33% and if this will improve patient survival. In this reveiw, we will discuss the experience with right heart sarcoma.

A Primary Cardiac Sarcoma With Features of a Desmoplastic Small Round Cell Tumor in an Adolescent Male

Malignant primary cardiac tumors are very rare.Desmoplastic small round cell tumors are also rare tumors and,when present, are generally found in the abdomen. We report a case of an adolescent male who presented with chest pain,abdominal pain, and difficulty in breathing, who was found to have a primary cardiac sarcoma with several characteristic features of a desmoplastic small round cell tumor.

Les tumeurs cardiaques primitives malignes de l’enfant et de l’adolescent

Primary heart tumors are uncommon in children. The majority of them are benign, with only 10% malignant. Among malignant cardiac tumors, sarcoma (rhabdomyosarcoma, angiosarcoma, synovial sarcoma) and lymphoma (Burkitt's lymphoma, large B-cell lymphoma, lymphoblastic lymphoma) predominate. There are few published pediatric series on malignant primary cardiac tumors. We report here 3 observations of primary malignant cardiac tumors, 2 cases of sarcoma (angiosarcoma and synovial sarcoma) and 1 case of Burkitt's lymphoma. A precise pathological diagnosis is necessary for the proper management of these patients. For sarcoma, treatment associates surgery and chemotherapy. Surgery should be as complete as possible because of the lack of chemotherapy sensitivity of some sarcomas, mainly angiosarcoma and synovial sarcoma. Therefore, the prognosis of cardiac sarcoma remains poor. For primary cardiac lymphoma, management should not be different from lymphoma in other locations. Chemotherapy is the main treatment, and surgery has to be used only when complications occur. Prognosis depends on histology and not lymphoma location, and so is better than the prognosis for sarcoma.

Characterization and Management of Cardiac Tumors

Cardiac tumors are infrequent clinical entities with an autopsy frequency ranging from 0.001% to 0.030%. The occurrence of metastatic cardiac tumors has been reported a 100-fold more commonly than primary lesions. Three quarters of primary cardiac tumors are benign; approximately half of these are cardiac myxomas, and the rest are lipomas, papillary fibroelastomas, and rhabdomyomas. Among malignant primary cardiac tumors, the most reported are those histopathologically considered as undifferentiated, followed by angiosarcomas and leiomyosarcomas. Traditionally, cardiac tumors have been identified as curious autopsy findings resulting in a literature paucity of large clinical series, therefore, providing knowledge mostly based on case report collection. However, recent technological advances in noninvasive imaging modalities such as echocardiography and cardiac magnetic resonance imaging (MRI) have resulted in a rapid acquisition of real-time heart images with high spatial and temporal resolution and an excellent tissue characterization of the tumor. This consequent earlier, more frequent, and more complete assessment of cardiac tumors before significant symptoms develop has challenged cardiologists, cardiac anesthesiologists, and surgeons to create a tailored referral pattern and approach.

Primary cardiac tumors: a clinicopathologic evaluation of four cases

We report the clinical, pathological, and immunohistochemical features of four primary malignant cardiac tumors identified at the Department of Pathology, Rigshospitalet, Denmark. A panel of immunohistochemical markers for classification is proposed. Between 2000 and 2008, four patients with malignant cardiac tumors were treated at our hospital. We retrospectively reviewed the medical records and evaluated the patient characteristics and treatment. Three patients presented with severe dyspnea; one patient presented with chest pain. Transthoracic echocardiography demonstrated, in all four cases, abnormal masses in the atria. The cases were, based on morphological features and immunoprofile, classified as myogenic sarcoma (two cases), undifferentiated pleomorphic sarcoma, and leiomyosarcoma. Three of the patients received orthotopic heart transplantation. One patient survived 6.5 years after the diagnosis, and two patients are still alive 2 and 3 years after being diagnosed, respectively. All four cases were sarcomas. A limited number of immunohistochemical markers can be used in order to define a specific line of differentiation. In this small study, three of the patients were offered orthotopic heart transplantation, and the survival times were generally longer than in most series.

Large Obstructive Cardiac Myxofibrosarcoma Is Nearly Invisible on Transthoracic Echocardiogram

Malignant primary cardiac tumors are exceptionally rare, and the most common of these are sarcomas. We present a case of a patient who was found to have a large mass in the left atrium causing obstruction of mitral inflow. Only selected views on a transthoracic echocardiogram reveal the mass and its hemodynamic effect. Although the mass was sizeable, it could be easily overlooked on cursory review of the echocardiogram. After surgical resection, the mass was proven to be a myxofibrosarcoma. We review the etiologies and possible solutions for the imperfect sensitivity of transthoracic echocardiography for cardiac masses.

Cardiac Angiosarcoma Presenting With Right Coronary Artery Pseudoaneurysm

ANGIOSARCOMA OF THE HEART, although rare, is the most common primary malignant cardiac tumor. 1 Yu K. Liu Y. Wang H. et al. Epidemiological and pathological characteristics of cardiac tumors: A clinical study of 242 cases. Interact Cardiovasc Thorac Surg. 2007; 6: 636-639 Crossref PubMed Scopus (121) Google Scholar Prognosis is poor regardless of treatment, although survival is longest when surgical resection is possible. 2 Putnam Jr, J.B. Sweeney M.S. Colon R. et al. Primary cardiac sarcomas. Ann Thorac Surg. 1991; 51: 906-910 Abstract Full Text PDF PubMed Scopus (262) Google Scholar , 3 Simpson L. Kumar S.K. Okuno S.H. et al. Malignant primary cardiac tumors: Review of a single institution experience. Cancer. 2008; 112: 2440-2446 Crossref PubMed Scopus (204) Google Scholar Diagnosis is usually delayed because this tumor is typically associated with a variety of nonspecific symptoms. 3 Simpson L. Kumar S.K. Okuno S.H. et al. Malignant primary cardiac tumors: Review of a single institution experience. Cancer. 2008; 112: 2440-2446 Crossref PubMed Scopus (204) Google Scholar , 4 Donsbeck A.V. Ranchere D. Coindre J.M. et al. Primary cardiac sarcomas: An immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology. 1999; 34: 295-304 Crossref PubMed Scopus (163) Google Scholar The authors describe a patient with metastatic cardiac angiosarcoma whose presentation before diagnosis included a pericardial hematoma as a result of a coronary artery pseudoaneurysm.

A rare case of primitive right atrium angio-sarcoma with favorable outcome, in a young female: Case report and literature review

Primary malignant cardiac tumors are extremely rare, but their associated mortality is very high. The clinical presentation is oñen variable and nonspecific; by the time symptoms appear, the tumor has usually evolved to a large intracardiac and paracardiac mass causing considerable hemodynamic involvement, regional invasión and distant dissemination. We report a 26 year-old female with a right atrium angiosarcoma with a surprisingly favorable outcome at 2 years follow-up after treatment with a combination of neoadjuvant chemotherapy and surgical resection. She presented with chest tightness, shortness of breath at effort, atypical chest pain, palpitations, asthenia, weight loss and profuse perspiration. Physical examination and irnaging diagnostic procedures, identified a pericardial effusion and a pathologic cardiac mass. The diagnosis of a malignant angiosarcoma was suggested afier computed tomography transthoracic and transesophageal echocardiography It was confirmed at surgery by a biopsy followed by histopathology and immunohistochemistry.

Early detection and efficient therapy of cardiac angiosarcoma due to routine transesophageal echocardiography after cerebrovascular stroke

Primary malignant cardiac tumors (cardiac angiosarcomas) are exceedingly rare. Since there are initially nonspecific or missing symptoms, these tumors are usually diagnosed only in an advanced, often incurable stage, after the large tumor mass elicits hemodynamic obstructive symptoms. A 59-year-old female presented with symptoms of cerebral ischemia. A computed tomography (CT) scan showed changes suggestive of stroke. Transesophageal echocardiography revealed an inhomogeneous, medium-echogenic, floating mass at the roof of the left atrium near the mouth of the right upper pulmonary vein, indicative of a thrombus. At surgery, a solitary tumor was completely enucleated. Histologically, cardiac angiosarcoma was diagnosed. The patient received adjuvant chemotherapy and was free of symptoms and recurrence of disease at 14 months follow-up. Due to the fortuitous appearance of clinical signs indicative of stroke, cardiac angiosarcoma was diagnosed and effectively treated at an early, nonmetastatic, and therefore potentially curable stage. Although cardiac angiosarcoma is a rare disease, it should be taken into consideration as a potential cause of cerebral embolic disease.

Epidemiological and pathological characteristics of cardiac tumors: a clinical study of 242 cases

The purpose of this study was to review the current clinical experience and pathological characteristics of cardiac tumors. We retrospectively reviewed 33,108 consecutive cases of cardiac operations performed at our institution from October 1996 to March 2005. There were 242 cases confirmed histologically as cardiac tumors. Among them, 234 patients were diagnosed with primary heart tumors, revealing a prevalence of 0.71% among the corresponding period cardiac operations. Of primary cardiac tumors, the incidence of benign neoplasm was much higher than malignant masses (90.6% vs. 9.4%, P<0.01). The most common benign cardiac tumor was myxoma (86.8%). Mesenchymoma and angiosarcoma were the most common primary malignant cardiac tumors. The prevalence rates of cardiac tumors were quite different among age groups. Rhabdomyoma and fibroma are prone to children. The number of female patients with myxoma was higher than that in male (P<0.01). Myxomas have a special predilection for the left atrium (93.5%). Benign non-myxoma tumors are more likely to occur in the ventricular (64.3%). The primary malignant tumors have a tendency to be of multi-center origination (23%). All the secondary cardiac tumors were located in the right side of the heart. This study, using a relatively large sample, reveals the clinical incidences and pathological characteristics of various cardiac tumors in the Chinese population.

Exertional Dyspnea in a Soldier

Cardiac myxomas may present with a broad range of clinical features. There is a classic triad of features that includes obstructive cardiac signs, embolic signs, and constitutional manifestations. All patients present with one or more symptoms from this triad, but diagnosis may be elusive due to the nonspecific nature of some of these features. Although myxomas are uncommon and benign, the diagnosis must be considered when signs and symptoms suggest it due to the high associated morbidity and mortality. The size, character, location, and mobility of the tumor determine the type and severity of symptoms. Echocardiography is the most important tool used to establish the diagnosis, but CT and MRI may provide additional information in some cases. Prompt surgical resection of the tumor is the appropriate treatment. The overall prognosis is excellent, but patients should be followed due to the risk of recurrence.

Cardiac rhabdomyosarcoma

Primary malignant cardiac tumors are rare entities and rhabdomyosarcoma accounts 20% of these lesions. We presented a female patient with loss of appetite and loss of weight. She also had symptoms of heart failure and bilateral pleural effusions. A mobile tumor in the left heart with the entrance from the left atrium to left ventricle during diastole was seen by transthoracic echocardiography. The tumor was extirpated in total, histologic diagnosis was rhabdomyosarcoma, and the patient died after 6 months. Primary malignant cardiac tumors can simulate heart failure or systemic disorders. Cardiac rhabdomyosarcomas have a poor prognosis with the average survival of 6 months after the occurrence of symptoms and making a diagnosis. In cardiac insufficiency, differential diagnostics should be used to consider cardiac tumors that could be with certainty confirmed or excluded by echocardiography.

Primary Cardiac Osteosarcoma in a Young Man with Severe Congestive Heart Failure

A primary cardiac malignant tumor is very rare; its prevalence is only 0.002-0.28%. Among most malignant tumors, angiosarcoma, leiomyosarcoma, and mesothelioma occupy the majority. A cardiac osteosarcoma is extremely rare: to our knowledge, only 36 cases have been reported worldwide. We present a 22-year-old case featuring severe congestive heart failure. Hemodynamically the tumor led to significant obstruction of the mitral valve. The patient underwent an emergency resection operation, but multiple metastases occurred. Though the characteristics still remain unclear because of the low prevalence, it is very important that these tumors be distinguished from benign tumors because of early resection operation.

Malignant cardiac and pericardial neoplasms: 16-year surgical experience and follow-up

Aims: The literature on long-term follow-up of malignant cardiac and pericardial neoplasms is limited because of their rare occurrence and there is no comparison in the follow-up between patients with primary malignant cardiac tumors (PMCT), primary malignant paracardial tumors (PMPT), and metastases to the heart and mediastinum

Cardiac angiosarcoma: case report and review of the literature

Angiosarcoma of the heart, the most common primary malignant cardiac tumor in adults is known to carry a dismal prognosis. The diagnosis is often delayed because of the nonspecific clinical presentation. Symptoms are determined by the size and location of the tumor. Echocardiography has become the primary diagnostic technique because of its high degree of accuracy, noninvasiveness, and cost effectiveness. Complete surgical resection is required for improved survival. Conventional postoperative chemotherapy does not appear to modify the clinical course. We report a case of cardiac angiosarcoma with a large mural mass infiltrating the right atrial and ventricular walls and critically review the pertinent literature.

Herztumoren: Magnetresonanztomographie und Mehrschicht-Spiral-CT

Transthoracic echocardiography is usually the initial diagnostic test in patients with a suspected cardiac mass. However, this technique is restricted by its small field of views and insufficient acoustic window in some patients. Magnetic resonance imaging (MRI) and, since its introduction, multislice spiral computed tomography allow for detailed delineation of intra and pericardiac tumors, their extent, and their influence on cardiac function. Primary benign and malignant cardiac tumors have several characteristic features in MR imaging. Assessment of such features may narrow down the differential diagnosis or even allow for reliable diagnosis in selected cases. Many such features can also be assessed using MSCT. This article provides an overview of examination protocols of MRI and CT for cases in which a cardiac mass is suspected and describes the appearance of primary and secondary cardiac masses as well as intracavitary thrombi.

H�morrhagisch unspezifischer Perikarderguss als Vorbote eines H�mangiosarkoms des rechten Herzens

A 45-year old woman presented at our hospital with intermittent fever in the last three weeks and progressive exertional dyspnea. In addition she suffered from physical asthenia. Seven months ago the patient was treated for symptomatic pericardial effusion in our clinic. Physical examination revealed a pale skin color, epigastric pain on palpation and intense exertional dyspnea. Laboratory tests indicated microcytic anemia and high levels of LDH and CRP. The diagnosis of a large tumor of the right atrium and ventricle was based on the transthoracic echocardiogram and thoracic CT scan. The extent of the right atrial and ventricular mass of 9 x 8 cm was detected by trans-esophageal echocardiography. The thoracic and abdominal CT scan showed multiple nodular infiltrates in the basal lung fields and a cystic tumorous mass in the liver. The tumor developed in the short time of 7 months. Half a year ago, the transthoracic echocardiographic exam showed pericardial effusion with normal cardiac size, normal left ventricular function and no evidence of right ventricular outflow obstruction. No cardiac masses were observed. At that time pericardiocentesis was performed, but the pericardial fluid was cytologically negative for tumor cells. The diagnosis of angiosarcoma was made after surgical excision of the tumor and histological examination. There was only the possibility of a palliative therapy, because of the existence of pulmonary and liver metastases. Primary malignant cardiac tumors are rare and their prognosis is very poor. The heart angiosarcoma is often disseminated into the lungs and the liver at the time of clinical presentation. This case indicates that hemorrhagic nonspecific pericardial effusion, negative for tumor cells, can appear any time before a heart angiosarcoma is detectable by echocardiography.

Calcified Left Ventricular Mass: Unusual Clinical, Echocardiographic, and Computed Tomographic Findings of Primary Cardiac Osteosarcoma

Calcified Left Ventricular Mass: Unusual Clinical, Echocardiographic, and Computed Tomographic Findings of Primary Cardiac Osteosarcoma