Primary Cardiac Tumors Research Articles

Complete resolve of primary cardiac sarcoma by chemotherapy: A case report

Primary cardiac tumors (PCT) are rare with high mortality rate and low survival. There are several treatment approaches in the PCT management. Complete surgical resection is the main base of treatment and there is disagreement on adjuvant chemotherapy or radiotherapy after surgical resection. In this case report, complete remission of the relapsed non-metastatic cardiac synovial sarcoma was observed after salvage chemotherapy and complete surgical resection.

Primary cardiac tumours: how well can prenatal diagnosis "predict" postnatal outcome?

Primary foetal cardiac tumours are rare congenital malformations. They can cause a flow obstruction, arrhythmias and can lead to cardiac failure, hydrops or death. Postnatal management is based on patient´s clinical and hemodynamic impairment. We retrospectively reviewed data from 2009-2019 from our gynaecology clinic and also data regarding postnatal follow-up from our partner paediatric institution. In this period, we diagnosed six cases with foetal cardiac tumours. In four cases, multiple rhabdomyomas were present. Three patients did not have serious complications pre- or postnatally. In one case, tumours were obliterating both the inflow and the outflow of the left ventricle. The child died at three months of age. Tuberous sclerosis was confirmed in all the cases with rhabdomyomas. One child had a fibroma filling the left ventricle. Despite an uneventful prenatal period, the patient got postnatally symptomatic. Tumour was considered inoperable and the child died at the age of five months. In one case a single right ventricular unspecified tumour was diagnosed, without any complications. Prognosis closely depends on early diagnosis, clinical manifestations and the possibility of surgical tumour removal if necessary. In confirmed rhabdomyomas, tests for tuberous sclerosis are mandatory (Tab. 1, Fig. 2, Ref. 18). Text in PDF www.elis.sk Keywords: rhabdomyoma, fibroma, prenatal diagnosis, ultrasound, tuberous sclerosis.

Surgical Treatment of Cardiac and Pulmonary Sarcomas

Primary cardiac tumors have an autopsy incidence of between 0.02% and 0.04% or about 200 tumors per 1 million autopsies. Approximately 75% of these neoplasms are benign and 25 % malignant. Primary cardiac sarcomas (PCS) make up over 75% of the primary malignant tumors. The rarity of primary cardiac tumors has led to a minority of surgeons and even few institutions having substantial experience with their treatment. Surgical resection of primary cardiac sarcoma is the mainstay of therapy but is less well understood and far more difficult. Additionally, the preoperative evaluation, preparation and decision making about the ability to achieve complete resection can be complex and has not been standardized. We separate sarcoma into right heart, left heart and pulmonary artery sarcoma as anatomic location often dictates the clinical presentation and the surgical options available. This manuscript presents our approach to the evaluation, preparation and surgical techniques required for treating these primary cardiac sarcomas. Primary cardiac tumors have an autopsy incidence of between 0.02% and 0.04% or about 200 tumors per 1 million autopsies. Approximately 75% of these neoplasms are benign and 25 % malignant. Primary cardiac sarcomas (PCS) make up over 75% of the primary malignant tumors. The rarity of primary cardiac tumors has led to a minority of surgeons and even few institutions having substantial experience with their treatment. Surgical resection of primary cardiac sarcoma is the mainstay of therapy but is less well understood and far more difficult. Additionally, the preoperative evaluation, preparation and decision making about the ability to achieve complete resection can be complex and has not been standardized. We separate sarcoma into right heart, left heart and pulmonary artery sarcoma as anatomic location often dictates the clinical presentation and the surgical options available. This manuscript presents our approach to the evaluation, preparation and surgical techniques required for treating these primary cardiac sarcomas.

Management of Cardiac Paragangliomas

Primary cardiac tumors are uncommon with 75% being benign and 25 % malignant. Most benign tumors are relatively easy to manage surgically. There are however, benign tumors that can present management and technical challenges for surgical care. Paraganglioma is one of these tumors. These tumors may present as cathecholamine producing tumors requiring pre-operative preparation for safe resection and tend to be large and in difficult anatomic locations to remove. This manuscript details our approach to these tumors. Primary cardiac tumors are uncommon with 75% being benign and 25 % malignant. Most benign tumors are relatively easy to manage surgically. There are however, benign tumors that can present management and technical challenges for surgical care. Paraganglioma is one of these tumors. These tumors may present as cathecholamine producing tumors requiring pre-operative preparation for safe resection and tend to be large and in difficult anatomic locations to remove. This manuscript details our approach to these tumors.

Surgical Treatment of Benign Cardiac Tumors

Myxoma and papillary fibroelastoma are the most common benign cardiac tumors accounting for the majority of the benign tumors a surgeon will see. (2) Myxomas occur most commonly in the left atrium but can occur in any cardiac chamber and represent over 50% of the benign tumors. Fibroelastomas are the second most common benign tumors and generally involve the cardiac valves but can occur within the chambers. Surgical resection is the appropriate approach for myxoma and most fibroelastomas and the surgical techniques needed have been well established. This manuscript presents our approach to the evaluation, preparation and surgical techniques required for treating these primary benign cardiac tumors. Myxoma and papillary fibroelastoma are the most common benign cardiac tumors accounting for the majority of the benign tumors a surgeon will see. (2) Myxomas occur most commonly in the left atrium but can occur in any cardiac chamber and represent over 50% of the benign tumors. Fibroelastomas are the second most common benign tumors and generally involve the cardiac valves but can occur within the chambers. Surgical resection is the appropriate approach for myxoma and most fibroelastomas and the surgical techniques needed have been well established. This manuscript presents our approach to the evaluation, preparation and surgical techniques required for treating these primary benign cardiac tumors.

Successful surgical management of a rare combination of intrapericardial teratoma and thymoma in an infant

Primary cardiac tumors are rare, accounting for <0.2% of all childhood tumors. They can be diagnosed prenatally. Intrapericardial teratoma is a rare benign tumor that presents either due to the mass effect of the tumor or secondary pericardial effusion. Thymus is an important part of the immune system in the pediatric age group. Thymic lesions are rare causes of anterior mediastinal pathology. Their occurrence in children is rarer, nevertheless knowledge about their pathologies helps in clinching the correct diagnosis. We report a case of combined intrapericardial teratoma and thymoma that has not been reported previously in the literature.

Cardiac myxoma as cytokine producing tumour: A review

Cardiac myxoma is the most common primary cardiac tumour. Many cytokines participate in the pathophysiology and growth of cardiac myxoma. Inflammatory cytokines, including interleukin-1, interleukin-4, interleukin-6, interleukin-8, interleukin-12, tissue necrosing factor-α, and interferon-γ, contribute to the development of inflammation and inflammation-related symptoms and further affect tumour growth. Growth factors, including vascular endothelial growth factor, basic fibroblast growth factor, insulin-like growth factor 1, and epidermal growth factor, contribute to angiogenesis and tumour growth and interfere with the inflammatory response. Recently, we reported a cardiac myxoma whose cells were positive for interleukin-1β and hemopoietic factor granulocyte colony-stimulating factor in addition to interleukin-6. This review summarises the current knowledge of cardiac myxomas as cytokine-producing tumours.

Primary Cardiac Rhabdomyosarcoma in a Child—A Case Report

Unlike benign primary cardiac tumors like rhabdomyomas and fibromas, primary malignant tumors are extremely rare in children with very few cases reports in the literature so far. Rhabdomyosarcoma is a rare primary malignant tumor in children with most reported cases occurring in the right heart. We report a 15-month-old child with a primary rhabdomyosarcoma arising from the left ventricle and presenting with cardiac tamponade and circulatory failure. He underwent a diagnostic sternotomy and palliative chemotherapy but succumb to the disease later.

Clinical Significance of Angiographically Detectable Neovascularity in Patients with Cardiac Myxoma

Background: Myxomas are the most common primary cardiac tumors. Angiographically detectable neovascularity (ADN) of myxoma is increasingly being reported as a result of the use of coronary angiography (CAG) to detect coronary artery disease. However, the clinical significance of these findings is not fully understood. Methods: We enrolled 59 patients with cardiac myxoma who also underwent CAG between January 2013 and October 2018. Patients were followed up for a mean of 28.9 months (range 1–69 months). The clinical features, echocardiography measurements, pathological examination findings, CAG results, and outcomes during follow-up were compared between patients with ADN and patients without ADN. Results: ADN was found in 25 patients (42.4%). The arteries feeding the ADN included the right coronary artery (n=15), the left circumflex coronary artery (n=7), and both arteries (n=3). The patients with ADN had a higher proportion of eosinophils (3.2% vs. 2.2%, P=0.03) and higher low-density lipoprotein cholesterol level (2.7 mmol/L vs. 2.2 mmol/L, P=0.02). Myxoma pedicles were more likely to be located in the interatrial septum in patients with ADN (96% vs. 73.5%, P=0.02). No significant correlation was observed between the groups in clinical manifestations, atrial arrhythmia, myxoma size, cardiac chamber size, left ventricular ejection fraction, and the prevalence of complication with coronary artery disease [16% in the ADN group (n=4) vs. 20.6% in the non-ADN group (n=7), P=0.66]. However, patients with ADN tended to have a lower incidence of major adverse cardiac and cerebrovascular events on long-term follow-up (0% vs. 14.7%, P=0.07). Conclusion: CAG-detected ADN in patients with cardiac myxoma is associated with a borderline lower rate of major adverse cardiac and cerebrovascular events.

The Rapid Growth Rate of a Left Atrial Myxoma Occurring Between two TOEs: A Case Report and Literature Review

Rare as they are, left atrial myxomas are the most common primary cardiac tumour. There is limited evidence around their growth rate, yet an increasing number of reports have illustrated that their growth may be faster than previously assumed. Our Case details a healthy 32-year-old female who suffered two debilitating ischaemic cerebral infarcts and was later found to have a rapidly growing left atrial myxoma. We conducted a literature search of previously documented left atrial myxomas where a growth rate was either reported or could be derived from the information provided. The minimum growth rate of our cases’ atrial myxoma was calculated at 7.7mm per month over the course of four and a half months. To the best of our knowledge, this is the second-fastest growing atrial myxoma ever reported, and the fastest documented growth rate of a left atrial myxoma that has been derived between two transoesophageal echocardiograms (TOEs), the gold standard in sonographic cardiac imaging. The true maximum growth rate of an atrial myxoma will likely remain undiscovered, however our Case provides added weight to the evidence that left atrial myxomas can rapidly evolve over a short space of time. Suspicion remains as to whether this left atrial myxoma may be a recurrence of an initial left atrial myxoma that fragmented and precipitated the two initial cerebral infarcts.

Characterizations of Ischemic Stroke Complications in Cardiac Myxoma Patients at a Single Institution in Eastern China.

BackgroundCardiac myxoma is the most common primary cardiac tumor. Even though it rarely causes a stroke, it is an important risk factor. Here, we compared our clinical experience in managing myxoma patients who developed stroke complications with those who did not present with this condition at the First Affiliated Hospital of Wenzhou Medical University.Patients and MethodsThe medical records were reviewed of 160 cardiac myxoma patients who were treated in our facility from January 2006 to December 2019. They were separated into either a stroke group or non-stroke group.ResultsCardiac obstructive symptoms, embolic events and constitutional symptoms were observed in 92 (57.7%), 25 (15.6%) and 18 (11.2%) patients, respectively. Among 23 cardiac myxoma ischemic stroke patients, hypoesthesia (60.9%), hemiparesis (56.5%) and facial paresis (47.8%) were the three most common neurological symptoms. The middle cerebral artery (82.6%) was the most commonly affected vessel, whereas 73.9% of the ischemic patients had multiple stroke lesions. Binary logistic regression analysis showed that coronary heart disease and tumor sizes were independently associated in the stroke group (p <0.05). The 10 years cumulative survival rate was 87.9% for all patients after surgical intervention. There was no significant difference in the 10 years cumulative survival rate between the two groups (80.0% vs 88.9%, p =0.274 > 0.05).ConclusionThe three most common neurological symptoms (hypoesthesia, hemiparesis and facial paresis), the middle cerebral artery and multiple lesions involvements were the definitive markers of patients afflicted with cardiac myxoma stroke. Small tumor sizes were independently associated with these patients. Surgical resection is a relatively safe procedure for treating both the stroke and non-stroke patients.

CARDIACMYXOMA: THE PARADOXBETWEEN ITSBENIGNITY AND ITSDRAMATIC COMPLICATIONS: 5 CASE REPORTS AND LITERATUREREVIEW

Cardiacmyxoma are the most common benign primary cardiactumors that can lead to many complications as described in literature. It is a benigntumor but her complications are dramaticbecause of herlocalization and hisrisk of embolization in otherorganswhich put the vital prognosisatrisk. We report 5 cases withdifferentclinicalsymptoms. We illustrate through these cases the embolic and obstructive complications of cardiacmyxoma and weinsist on the interest of an urgent surgicaltreatment.

Left Atrial Myxoma Misdiagnosis as Infective Endocarditis: A Case Report

Introduction: Myxomas, as primary cardiac tumors, are rare neoplasms manifesting with constitutional, obstructive, and embolic symptoms. Systemic signs and symptoms make infective endocarditis one of the most common differential diagnoses. Nonspecific systemic signs and symptoms of myxomas can cause great challenges in management and treatment. Case Presentation: A 21-month-old boy was admitted to our center with a high-grade fever and gastrointestinal symptoms, but no significant respiratory complaints were noted. A history of lower limb pain and morning claudication was also present. The patient had leukocytosis with an elevated erythrocyte sedimentation rate (ESR) of two months' duration. Other acute phase reactants were detected despite previous antibiotic therapy; however, his blood culture result was negative. In echocardiography, multiple masses were seen in the left atrium near the mitral valve apparatus. The diagnosis of infective endocarditis was made, and intravenous antibiotics were prescribed for up to two weeks. He then underwent surgery because of poor response to medical treatment and renal complications. During surgery, a small mass was seen in the left atrium, and two other masses were within the mitral chordae. The histopathology report confirmed atrial myxoma. Shortly after surgical resection of the tumors, the patient's symptoms improved, and inflammatory indices returned to normal. Conclusions: Constitutional manifestations of myxoma render it reasonable to consider it as a differential diagnosis of infective endocarditis. Obtaining a complete clinical history and using imaging tools are necessary for the correct diagnosis and prevention of complications of inappropriate treatment.

Extensive pulmonary metastases in young boy with primary cardiac angiosarcoma: a case report.

BackgroundMalignant primary cardiac neoplasms are rare and primary cardiac angiosarcoma is the most common and aggressive subtype. It most commonly presents in middle-aged males and due to its non-specific clinical presentation, the diagnosis is often delayed until advanced disease is already present. Clinical presentation is determined by manifestations of local infiltration or metastatic disease and making an early diagnosis is extremely challenging.Case summaryA 15-year-old previously healthy boy was admitted to the emergency department with a history of pathological weight loss and functional decline. The patient was found to have a left-sided pneumothorax as well as bilateral diffusely spread pulmonary nodules on plain chest radiograph. Computed tomography chest confirmed widespread pulmonary metastases and a right atrial filling defect. Echocardiography revealed a right atrial tumour and transvenous endomyocardial biopsy of the tumour was done under fluoroscopic and echocardiographic guidance. A diagnosis of primary cardiac angiosarcoma was made. The patient demised shortly after presentation.DiscussionPrimary cardiac angiosarcoma is rare and even more so in patients as young as the case described. The diagnostic process poses several challenges to the clinician, of which the obtaining of a histological sample is one. This case report demonstrates aspects both unique and typical of this rare disease. It also describes an effective option for obtaining tissue for a histological diagnosis in patients whose clinical condition may not allow biopsy under general anaesthesia.

Multiple Primary Cardiac Malignant Peripheral Nerve Sheath Tumors in the Left Atrium

Malignant peripheral nerve sheath tumors are rare sarcomas of the heart. Herein, we report the case of a 24-year-old man who complained of dyspnea, cough, and upper left back pain. He was found to have multiple primary heart tumors obstructing the right superior pulmonary vein in the left atrium, which were diagnosed as malignant peripheral nerve sheath tumors. The patient underwent successful resection of the tumors and immunohistochemistry was utilized for diagnosis.

Minimally invasive approaches to primary cardiac tumors: A systematic review and meta-analysis.

Cardiac tumors are rare conditions. The vast majority of them are benign yet they may lead to serious complications. Complete surgical resection is the gold standard treatment and should be performed as soon as the diagnosis is made. Median sternotomy (MS) is the standard approach and provides excellent early outcomes and durable results at follow-up. However, minimally invasive (MI) is gaining popularity and its role in the treatment of cardiac tumors needs further clarification. A systematic literature review identified 12 candidate studies; of these, 11 met the meta-analysis criteria. We analyzed outcomes of 653 subjects (294 MI and 359 MS) with random effects modeling. Each study was assessed for heterogeneity. The primary endpoints were mortality at follow-up and tumor relapse. Secondary endpoints included relevant intraoperative and postoperative outcomes; tumor size was also considered. There were no significant between-group differences in terms of late mortality (incidence rate ratio [IRR]: MI vs. MS, 0.98 [95% confidence interval [CI]: 0.25-3.82], p = .98). Few relapses (IRR: 1.13; CI: 0.26-4.88; p = .87) and redo surgery (IRR: 1.92; 95% CI: 0.39-9.53; p = .42) were observed in both groups; MI approach resulted in prolonged operation time but that did not influence the clinical outcomes. Tumor size did not significantly differ between groups. Both MI and MS are associated with excellent early and late outcomes with acceptable survival rate and low incidence of recurrences. This study confirms that cardiac tumor may be approached safely and radically with a MI approach.

Primary Cardiac Tumors in Children: An 8-Year-Experience in a Single Center

Background: Cardiac neoplasms are not common in the pediatric population, and most of the reported cases in the literature are benign. Despite their benign nature, however, cardiac neoplasms may lead to hemodynamic instability and arrhythmias. Objectives: Our study aimed to review the natural history, diagnosis, pathological features, and outcome of pediatric patients with cardiac tumors presenting to a tertiary pediatric cardiac center over an 8-year period. Methods: This retrospective observational study reviewed and analyzed the hospital records and data of pediatric patients diagnosed with cardiac tumors over an 8-year period (2007 - 2015). Results: During the period covered by the study (2007 - 2015), a total of 62975 pediatric in and out patients were referred to our tertiary cardiac center for cardiac evaluation. Cardiac tumors were identified in 41 patients, in 35 of whom tumor types were recognized. Rhabdomyomas were the most common tumors, followed by fibromas and myxomas. Fourteen patients underwent surgical management (either total or partial resection), and 4 patients received chemotherapy. Associated congenital heart diseases, except for the patent foramen ovale, were detected in12 patients. Conclusions: While almost all our findings were compatible with previous data, we had a comparatively large number of undetermined tumors. Therefore, a better understanding of the imaging characteristics of cardiac tumors is needed for a better diagnosis of tumor types via noninvasive modalities.

Left-sided primary cardiac lymphoma: a case report

BackgroundPrimary cardiac lymphoma (PCL) is a rare neoplasm, defined as lymphoma with the main bulk localized in the heart; diffuse large B cell lymphoma (DLBCL) is the most common type. It usually involves the pericardium and the right heart and has a poor prognosis with a median survival of less than 1 year.Case presentationA 62-year-old female presented to the emergency department for palpitations and recent-onset dyspnea. The echocardiogram showed a round-shaped mass (33 x 32 mm) in the left atrium, a mild circumferential pericardial effusion, and a hyperechogenic mass at the level of the atrio-ventricular junction. A thoracic CT scan demonstrated the presence of a bulky mediastinal mass, strictly attached to the left heart and infiltrating its posterior wall, the left pulmonary veins, and the inferior lobar bronchus. The patient underwent CT-guided biopsy, and the diagnosis of double-expressor DLBCL was made. Given the potential risk of heart rupture during chemotherapy, the first cycle of R-CHOP was performed in an in-hospital setting, with initial benefit. After completing 6 cycles of R-CHOP, imaging studies showed rapid progression of the disease; the patient was started on the salvation protocol R-DHAOX but died of septic shock 10 months later.ConclusionsPCL is rare and accounts for less than 2% of primary cardiac tumors; double-expressor DLBCL carries a poor prognosis.As in most cases, the diagnosis was made after the onset of nonspecific symptoms (dyspnea) but—despite the strong predilection for right heart involvement reported in literature—our patient had a predominant left atrial infiltration.

30-year follow-up of an unoperated left atrial myxoma: a case report.

BackgroundCardiac myxomas are the most common benign primary cardiac tumours. The natural history of left cardiac myxomas is thought to be of slowly growing tumours. Cardiac myxomas are a heterogeneous group with a variable growth rate. They present usually with stroke, valve obstruction, or non-specific symptoms. Surgical resection is the effective treatment.Case summaryThis case report describes a 56-year-old hypertensive and dyslipidaemic female, when she was admitted in January 1990, complaining of loss of appetite, aches, pains, and palpitations. Her workup included a transthoracic echocardiography and transoesophageal echocardiography, which showed a left atrial mass attached to the inter-atrial septum, highly suggestive of left atrial myxoma. She was referred for surgical removal of the left atrial mass. However, she was reluctant to undergo surgery as she felt better. The patient was followed-up for almost 30 years with the left atrial mass confirmed as left atrial myxoma by cardiac magnetic resonance imaging. The left atrial mass became smaller in size and more calcified.DiscussionCardiac myxomas are a group of heterogeneous tumours, thought to be slowly growing. The growth rate of cardiac myxomas prior to diagnosis is not well known, as the vast majority is treated with surgical resection immediately after diagnosis. Our case showed the natural progression of an unoperated smooth-surfaced left atrial myxoma followed-up for almost 30 years, which slowly became smaller and more calcified.

Edema agudo de pulmón en paciente con masa auricular izquierda ¿Qué podemos encontrar en la ecografía cardiaca transtorácica?

Los tumores cardiacos primarios son una entidad poco frecuente. Un 75% de éstos son de estirpe benigna y, entre ellos, el mixoma auricular es el más común, presentándose alrededor del 50%, con mayor prevalencia en mujeres de 13 a 80 años. Los síntomas del mismo son muy variados, desde pacientes asintomáticos hasta aquellos que han presentado embolismo, síncope, arritmia, insuficiencia cardiaca, astenia o anorexia entre otros. Presentamos el caso clínico de una paciente que se somete a intervención quirúrgica de mixoma auricular izquierdo y, el manejo anestésico necesario para abordar dicha cirugía.