Malignant Cardiac Tumors Research Articles

Large malignant cardiac tumor in a 7-year-old child

Primary malignant cardiac tumors are extremely rare in childhood and among them, sarcomas are most frequent. We present clinical, echocardiographic and magnetic resonance imaging findings of a 7-year-old child in whom an invasive malignant cardiac tumor was detected at the level of the right atrium and ventricle, with the presence of pulmonary metastases at the time of diagnosis.

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.

Unusual presentation of multiple recurrent cardiac sarcomas as acute acalculous cholecystitis caused by rapid expansion into the right cardiac chamber: report of a case

Sarcomas are rare malignant cardiac tumors and their prognosis is extremely poor, with a high recurrence rate despite extensive initial surgical resection. The development of therapeutic alternatives looks bleak for recurrent sarcomas, however, and complete surgical resection is crucial for any hope of survival even for the recurrent patients. We herein report the case of a 38-year-old male patient with multiple recurrent cardiac sarcomas causing acute acalculous cholecystitis 20months after the resection of the primary tumor.

MR first pass perfusion of benign and malignant cardiac tumours—significant differences and diagnostic accuracy

To determine the diagnostic value of magnetic resonance (MR) first pass perfusion in the differentiation of benign and malignant cardiac tumours. 24 patients with cardiac tumours (11 malignant, histopathological correlation present in all cases) were examined using MRI. In addition to morphological sequences a saturation-recovery T1w-GRE technique was implemented for tumour perfusion. The maximum relative signal enhancement (RSE[%]) and the slope of the RSE(t)-curve (slopeRSE[%/s]) of tumour tissue were assessed. A t-test was used to identify significant differences between benign and malignant tumours. Sensitivities and specificities were calculated for detection of malignant lesions and were compared with the sensitivity and specificity based on solely morphological image assessment. The RSE and slopeRSE of malignant cardiac tumours were significantly higher compared with benign lesions (p < 0.001 and p < 0.001). The calculated sensitivities and specificities of RSE and slopeRSE for identification of malignant lesions were 100% and 84.6% and 100% and 92.3%, respectively with cut-off values of 80% and 6%/s. The sensitivity and specificity for identification of malignant lesions on the basis of morphological imaging alone were 90.9% and 69.2%. With first pass perfusion, malignant cardiac masses can be identified with higher sensitivity and specificity compared with morphological image assessment alone.

Radiofrequency Catheter Ablation of Life-Threatening Ventricular Arrhythmias Caused by Left Ventricular Metastatic Infiltration

Metastases to the cardiac ventricles are rare and, unfortunately, often follow a rapidly fatal course.1,2 Occasionally, they cause symptomatic ventricular arrhythmias (VAs) for which limited therapeutic options exist, such as antiarrhythmic drugs, whereas the use of catheter ablation, to our knowledge, has never been reported to date. We present the case of a 27-year-old man with a metastatic tumor in the left ventricle and intractable malignant VAs. In February 2009, the patient had been diagnosed with a gluteal sarcoma and initially treated with combined chemotherapy (including anthracyclines) and radiation. Since December 2009, a mild reduction in left ventricular ejection fraction (45%) was detected, and since January 2010, frequent monomorphic premature ventricular contractions (PVCs) and nonsustained ventricular tachycardias (VTs) were documented. Treatment with amiodarone proved helpful, and no life-threatening VAs could be induced on standard electrophysiological study.3 Since April 2010, however, the patient suffered from multiple drug-refractory episodes of sustained VT and ventricular fibrillation, requiring several direct current shocks (Figure 1). His left ventricular ejection fraction had remained stable. Electrical storm resulted in immediate hemodynamic instability and dramatically affected his quality of life. The patient was referred to our institution, where multiple antiarrhythmic drug combinations were tested, both orally and intravenously, allowing a relative stabilization of cardiac rhythm. Cardiac MRI showed a T2-hyperintense, weakly gadolinium-enhanced area at the basis of the anterior papillary muscle, compatible with metastatic infiltration (Figure 2, online-only Data Supplement Movie 1). An oncology consultation hypothesized a life expectancy of <6 months. After about 2 weeks, the recurrence of multiple episodes of VT and ventricular fibrillation required intensive care treatment with deep sedation and assisted ventilation, but still VAs could not be controlled. Thus, a repeat electrophysiological evaluation and radiofrequency …

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.

Intracardiac Metastasis of Esophageal Squamous Cell Carcinoma: Report of a Case

We report a case of a 48-year-old man with a history of violent coughing fits and general fatigue underwent urgent surgery for cardiac tamponade, and who was later diagnosed with metastatic intracardiac squamous cell carcinoma of the esophagus. After admittance to Munakata Suikokai General Hospital, Fukuoka, Japan, echocardiography showed extensive pleural and pericardial effusion and a mass, 4 by 2 cm, with a solid echo pattern in the right ventricular cavity. The working diagnosis was primary malignant cardiac tumor of unknown origin with multiple metastases. To prevent sudden death due to obliteration of the outflow tract of the right ventricle, we performed urgent surgery for cardiac tamponade. Histological examination of the resected tumor revealed squamous cell carcinoma. Fiberoptic esophagoscopy showed hypertrophy of the esophageal wall and a submucosal tumor extending throughout the esophagus. Microscopic examination of the esophagus biopsy specimen showed moderately differentiated squamous cell carcinoma, the histology of which was similar to that of the resected tumor and cytology of pericardial effusion. The patient recovered and was able to return home for a few days; however, he was readmitted, and despite maximal supportive therapy, he died one month after the operation.

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.

Cardiac Epithelioid Leiomyosarcoma and the Role of Cardiac Imaging in the Differentiation of Intracardiac Masses

Primary malignant cardiac neoplasms are extremely rare and can be difficult to differentiate from other intracardiac masses. We present a case of left atrial epithelioid leiomyosarcoma and review the imaging modalities available to diagnose and characterize intracardiac masses. We demonstrate how multimodal imaging aided diagnosis and proved crucial to establishing the tumor's location, degree of invasion, and involvement of surrounding structures prior to surgical resection.

Primary Cardiac Angiosarcoma: Morphologically Deceptive Benign Appearance and Potential Pitfalls in Diagnosis

Cardiac angiosarcoma is the most common malignant primary cardiac tumour. We report a case of cardiac angiosarcoma with recurrent complex pericardial effusion. Histological diagnosis was elusive underscoring the potential difficulty in differentiating this tumour from normal vascular endothelium.

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.

Focal caseous mitral annular calfication: Evaluation with cardiac MRI

We present a case of a 63-year-old male with an early caseous form of mitral annular calcification, which was incidentally found on a routine echocardiogram and initially suspicious for a malignant cardiac tumor. We show the relevance of using cardiac MRI in the workup of this important differential diagnosis of cardiac tumors.

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.

Rare Cause for Sudden Right Heart Failure

Right heart failure occurs daily in clinical settings, but an underlying cardiac malignant tumor is very uncommon. We report a case of a 48-year-old man presenting only with palpitations and decompensated heart failure. Echocardiographic imaging revealed a large tumor of the right ventricle. Shortly after a putatively successful surgical approach, the patient was admitted again with heart failure symptoms. On reassessment, a complete relapse with multiple metastases could be seen. Generally, cardiac malignant tumors are diagnosed at a time-point when therapeutic options are very limited or even postmortem. Broad echocardiographic screening in patients with unspecific symptoms might be helpful to detect cardiac malignant tumors at early stages.