[Fatal outcome of complete resection of undifferentiated pleomorphic cardiac sarcoma : About a case]. / Évolution fatale d'une résection complète d'un sarcome cardiaque pléomorphe indifférencié : à propos d'un cas.

INTRODUCTION: Undifferentiated pleomorphic sarcoma (UPS) is a very rare malignant primary cardiac tumor with a poor prognosis. OBSERVATION: A 34-year-old woman complained of dyspnea evolving in a febrile context with altered general condition: Transthoracic echocardiography (TTE) revealed a left intra-atrial mass measuring 51/26 mm in diameter. A thoracic computed tomography (CT) revealed a mass occupying the left atrium measuring 42/28/34 mm, associated with two pulmonary metastases. The cardiac mass was resected and the postoperative suites were fatal. Histological diagnosis was UPS. CONCLUSION: We present an atypical case of invasive UPS, which was surgically resected and scheduled for radio-chemotherapy with a fatal outcome.

Rapid Development of Primary Right Atrial Angiosarcoma.

Cardiac angiosarcomas are rare and generally followed by a high level of metastasis with poor median survival outcomes. Echocardiograms, CT scans, and MRIs are the standard methods for finding sites of cardiac tumors; however, immunohistochemical confirmation is necessary for a definitive diagnosis of angiosarcoma. A 58-year-old male presented to the emergency room with one week of dyspnea on moderate exertion accompanied by chest pain and alleviated with rest. A workup done to evaluate mass found a single 5 x 3.5 x 4.8 cm mass heavily vascularized by the right coronary artery and left circumflex involving the free wall of the right atrium with no extension to the tricuspid valve. Surgical resection was performed, and immunohistochemistry was consistent with a primary cardiac angiosarcoma. An exudative fluid analysis on pericardial and pleural fluid analysis may warrant screening for malignancy more frequently in concurrence with a patient's history and presentation. Although the time from onset of symptoms to diagnosis of cardiac angiosarcoma is not well established, further investigation of such correlation may offer insight into survival post-treatment.

Rare Cardiac Tumor: Do We Know All About Cardiac Myxofibrosarcoma?

Primary cardiac tumors (PCTs) are less frequent and carry an incidence of 1.38 per 100,000 population per year. Myxofibrosarcomas are reported as one of the rarest forms of cardiac sarcomas, mostly with mesenchymal origin and located in the left atrium. Current research indicates an increase in median survival from 14 months to 36 months following complete resection and chemoradiotherapy. A 55-year-old Caucasian woman was admitted with brief self-resolving episodes of aphasia following migraine headaches for the past few months with associated exertional dyspnea and episodes of hypotension. Examination revealed a right-sided facial droop with cardiac murmur on auscultation. MRI brain was recommended which revealed a non-hemorrhagic infarct and multiple watershed infarcts. A transesophageal echocardiography revealed a large mass of around 5 cm in size located at the posterior wall of the left atrium causing mitral stenosis. The patient was initially managed conservatively and referred to cardiothoracic surgery and underwent a complete surgical resection. The histopathological report indicated the presence of primary cardiac sarcoma, and a postoperative positron emission therapy (PET) scan revealed no other foci of cancer further strengthening evidence of a primary cardiac pathology. This case represents a rare cardiac pathology presenting with non-cardiac symptoms.

Cardiac Rhabdomyomas Presenting with Critical Cardiac Obstruction in Neonates and Infants: Treatment Strategies and Outcome, A Single-Center Experience.

Cardiac rhabdomyomas are the most common benign pediatric heart tumor in infancy, which are commonly associated with tuberous sclerosis complex (TSC). Most rhabdomyomas are asymptomatic and spontaneously regress over time. However, some cases especially in neonates or small infants can present with hemodynamic instability. Surgical resection of the tumor, which has been the gold standard in alleviating obstruction, is not always possible and may be associated with significant morbidity and mortality. Recently, mammalian target of rapamycin inhibitors (mTORi) have been shown to be safe and effective in the treatment of TSC. We present the outcomes of neonates and an infant who received treatment for symptomatic rhabdomyomas at a tertiary cardiology center. Medical records were reviewed to obtain clinical, demographic, and outcome data. Six patients received interventions for symptomatic rhabdomyomas, median age at presentation was 1 day old (range from 1 to 121 days old), and 67% of the patients had a pathogenic mutation in TSC gene. One patient underwent surgical resection of solitary tumor at right ventricular outflow tract (RVOT) successfully. In the four patients with left ventricular outflow tract (LVOT) obstruction, two patients received combined therapy of surgical debulking of LVOT tumor, Stage I palliation procedure, and mTORi and two patients received mTORi therapy. One patient with RVOT obstruction underwent ductal stenting and received synergistic mTORi. Four of the five patients had good response to mTORi demonstrated by the rapid regression of rhabdomyoma size. 83% of patients are still alive at their latest follow-up, at two to eight years of age. One patient died on day 17 post-LVOT tumor resection and Hybrid stage one due to failure of hemostasis, in the background of familial factor VII deficiency. Treatment of symptomatic rhabdomyoma requires individualized treatment strategy based on the underlying pathophysiology, with involvement of multidisciplinary teams. mTORi is effective and safe in inducing rapid regression of rhabdomyomas. A standardized mTORi prescription and monitoring guide will ensure medication safety in neonates and infants with symptomatic cardiac rhabdomyoma. Although the majority of tumors responded to mTORi, some prove to be resistant. Further studies are warranted, ideally involving multiple international centers with a larger number of patients.

Cardiac angiosarcoma: a case report.

Angiosarcoma is the most invasive and malignant cardiac tumor and most commonly originates from the right atrium. Early diagnosis is essential, and echocardiography has an important role in diagnosis. This tumor grows aggressively, and metastases to other sites makes it difficult to control. Surgical treatment remains the best option for patients who do not respond to chemoradiotherapy. We herein report a case of a 17-year-old patient with cardiac angiosarcoma who presented with dyspnea, chest pain, dry cough, and fever. Although we considered the most probable diagnosis to be constrictive pericarditis, pathologic examination revealed a primary angiosarcoma originating from the pericardium. The patient underwent total pericardiectomy. However, despite receiving chemotherapy for 2 weeks postoperatively, she developed complications including leukopenia and eventually died of respiratory failure. Late diagnosis of angiosarcoma often occurs, resulting in progression to end-stage disease and a very poor prognosis. Therefore, a thorough understanding of this entity, knowledge of its pitfalls in management, and establishment of an accurate treatment guideline would help to develop a reliable and life-saving treatment approach for these patients.

Cardiac Angiosarcoma With Pulmonary Metastasis: A Rare and Challenging Case.

Cardiac angiosarcoma is a rare and aggressive malignant tumor arising from the endothelial cells of the heart. It accounts for only a small fraction of all cardiac neoplasms and has a poor prognosis. We present a challenging case of a 20-year-old student who presented exertional dyspnea, palpitation, and occasional chest discomfort. Her clinical picture, radiological and pathological investigations confirm the diagnosis of cardiac angiosarcoma with pulmonary metastasis. This case highlights the importance of early diagnosis and multidisciplinary management for improved patient outcomes.

Clinical presentation, diagnosis, and management of primary cardiac tumor: A case report.

BACKGROUND: Primary cardiac tumors are rare but have the potential to cause significant morbidity if not treated in an appropriate and timely manner. To date, however, there have been no studies examining the survival characteristics of patients who did not undergo surgical resection. CASE REPORT: We are presenting a case of a 61-year-old male admitted to our department due to "heart tumor''. He had 5+years previous history of type 2 diabetes was, and he took Metformin orally for a long time. Under extracorporeal circulation and general anesthesia, the heart lesion was removed under thoracoscopy. The tumor was about 5 cm ∗ 4 cm pale yellow color and sent for biopsy. Pathology report showed tumor like hyperplasia of fat and striated muscle tissues, some fat cells had atypical hyperplasia. CONCLUSIONS: For patients with malignant tumor, most of them had local invasion or had distant metastasis when the diagnosis was clear, and only conservative management could be done. Moreover, the prognosis of malignant tumor is poor, and the pathological morphology is diverse. The causes of death are due to widespread tumor metastasis, refractory heart failure, and various arrhythmias.

Case report: obstruction in the right ventricular outflow tract due to hemolymphangioma.

Cardiac masses commonly involve primary tumors, metastatic diseases, and nonbacterial thrombotic and infective endocarditis. myxomas are the most common primary tumors, accounting for 75%. Hemolymphangiomas are a group of congenital vascular and lymphatic malformations that originate from the mesenchyme, with an incidence rate of 0.12%-0.28% per year. Hemolymphangiomas have been found in the rectum, small intestine, spleen, liver, chest wall, and mediastinum but have not yet been reported in the ventricular outflow tract in the heart. Herein, we report a case of a hemolymphangioma tumor in the right ventricular outflow tract (RVOT). The tumor was successfully resected, and the patient was followed up for 18 months and did not exhibit tumor recurrence.

Primary Cardiac Tumor in the Left Atrium: A Diagnostic Challenge.

An elderly man presented to the emergency department with shortness of breath, peripheral edema, and significant weight loss. Blood tests revealed anemia and elevated inflammatory markers, and chest imaging showed a massive left pleural effusion. During hospitalization, he developed subacute cardiac tamponade, and pericardiocentesis was performed. Further imaging revealed a primary malignant cardiac tumor with extensive infiltration of the cardiac tissue, and biopsy was deemed impossible due to the tumor's location. The most likely diagnosis was angiosarcoma. The cardiac surgery team evaluated the case and considered it inoperable due to the tumor's extensive infiltration. The patient is currently under the regular care of a palliative care team. This case underscores the difficulties of diagnosing primary cardiac tumors, particularly in elderly patients with comorbidities. Despite advances in imaging and surgical techniques, the prognosis for malignant cardiac tumors remains poor.

Intracardiac biopsy of cardiac tumors with echocardiographic guidance: Case report.

Background: Primary cardiac tumors are very rare, and about 20-30% of them are malignant tumors. Since early signs of cardiac tumors are non-specific, diagnosis can be challenging. There is a lack of the recommended guidelines or standardized strategies for diagnosis and optimal treatment for this disease. As the definite diagnoses of most tumors are made by pathologic confirmation, biopsied tissue is essential in determining the treatment for patients with cardiac tumors. Recently, intracardiac echocardiography (ICE) has been introduced to assist biopsy procedures of cardiac tumors and it provides high-quality imaging. Case Description: Due to its low prevalence and variable presentation, cardiac malignant tumors usually are easily missed. Hereby, we report three cases of patients who presented with non-specific signs of cardiac disorder and was initially suspended diagnosis as lung infection or cancer. Under the guidance of ICE, cardiac biopsies were successfully on cardiac masses, giving critical data for diagnosis and treatment planning. No procedural complications were obtained in our cases. These cases are intended to highlight the clinical value and importance of ICE-guided biopsy of intracardiac mass. Conclusions: The diagnosis of primary cardiac tumors relies on the histopathological results. In our experience, using ICE for biopsy of an intracardiac mass is an attractive tool to increase diagnostic results and reduce the risk of cardiac complications associated with inadequate targeting of the biopsy catheters.

Surgical treatment of rare pediatric cardiac myxomas:12 years clinical experience in a single institution.

BACKGROUND: Primary cardiac tumors are rare, and cardiac myxoma (CM) accounts for the majority of these tumors. Most of the reports in the literature are case reports. This study summarizes our clinical experience in the surgical treatment of CM over the past 12 years. METHODS: We retrospectively analyzed the clinical data of 23 children with CM(8 boys, 15 girls; median age: 8.92 months, range: 2 years 5 months-12 years 9 months; body weight: 11-45 kg, median body weight: 28.21 kg) admitted to our hospital in the previous 12 years, and we statistically analyzed their clinical manifestations and surgical methods. RESULTS: 23 cases underwent myxoma excision under cardiopulmonary bypass(CPB). The follow-up period was 0.2 to 12.6 years (mean:7.2 years). Two patients could not be traced, and the follow-up completion rate was 91.30%. One patient (4.35%) died of myocardial infarction early after surgery with low continuous cardiac output. There were no cerebral embolism, acute heart failure, atrioventricular block and other related complications in 19 cases. A patient with cerebral infarction complicated with right hemiplegia recovered well after rehabilitation treatment. There was no recurrence of CM in 19 cases and all patients recovered after surgery. One patient relapsed 5 years after surgery, and no tumor recurrence was observed after the second surgery. Among the 20 long-term survivors, 13 (65.00%) were NYHA Class I patients and 7(35.00%) were NYHA Class II patients. CONCLUSIONS: Although CM in children is rare, it may cause cerebral infarction and other multi-organ embolism. Once CM is found and removed as soon as possible, it can reduce serious complications. If the complete resection is possible, surgery provides better palliation. Follow-up echocardiographic should be paid attention to after surgery.

Surgical resection of a giant cardiac angiosarcoma and reconstruction of involved right heart structures: A case report.

We present the case of a young woman without a medical history who presented with a giant right atrial, transtricuspid, and right ventricular mass and in a severe clinical state. Multimodal imaging raised the suspicion of primary cardiac angiosarcoma. Due to rapid hemodynamic and respiratory deterioration, we were forced to perform surgical removal of the mass with a concomitant reconstruction of the involved right heart structures, only 48 h after presentation. The postoperative course was uneventful, and the patient was discharged from the intensive care unit 2 days later. Radical surgical resection with reconstruction of the resected heart structures was the only possible salvage option for giant angiosarcoma, which led to hemodynamic instability. Followed by chemotherapy, this radical approach may prolong survival.

Anesthetic management of primary cardiac tumor in the coronary sinus and right atrium.

The patient presented with complete atrioventricular block and dyspnea. They had a primary cardiac tumor originating in the coronary sinus, a rare site of origin. It filled the sinus and involved the right atrium. The patient might have presented with complete atrioventricular block due to tumor invasion and respiratory distress due to elevated LVEDP as the tumor filled the coronary sinus. As for anesthesia management, in addition to the usual management, we observed CS obstruction and also considered myocardial protection methods. It is important to anticipate the risks and develop an appropriate anesthetic plan accordingly.

Primary Cardiac Angiosarcoma Presenting as Cardiac Tamponade.

Malignant primary cardiac tumors are rare. The most common presenting symptom is dyspnea, which is non-specific. These tumors pose a significant diagnostic challenge, which when coupled with rapid disease progression can result in significant morbidity and mortality. Appearance of cardiac masses on CT and echocardiography can be non-specific. Cardiac MRI can help delineate cardiac tumors but definitive diagnosis requires mediastinal exploration and biopsy. Treatment includes radical resection followed by radiotherapy and chemotherapy along with targeted therapy. Metastasis often precludes candidacy for surgery, therefore, early diagnosis is pivotal. We present a patient with primary cardiac angiosarcoma who initially presented with cardiac tamponade and at time of diagnosis was not a surgical candidate. We aim to bring greater awareness to malignant primary cardiac tumors in hopes of increasing diagnostic suspicion to facilitate earlier diagnosis and treatment intervention.

Multimodality Imaging of Benign Primary Cardiac Tumor.

Primary cardiac tumors (PCTs) are rare, with benign PCTs being relatively common in approximately 75% of all PCTs. Benign PCTs are usually asymptomatic, and they are found incidentally by imaging. Even if patients present with symptoms, they are usually nonspecific. Before the application of imaging modalities to the heart, our understanding of these tumors is limited to case reports and autopsy studies. The advent and improvement of various imaging technologies have enabled the non-invasive evaluation of benign PCTs. Although echocardiography is the most commonly used imaging examination, it is not the best method to describe the histological characteristics of tumors. At present, cardiac magnetic resonance (CMR) and cardiac computed tomography (CCT) are often used to assess benign PCTs providing detailed information on anatomical and tissue features. In fact, each imaging modality has its own advantages and disadvantages, multimodality imaging uses two or more imaging types to provide valuable complementary information. With the widespread use of multimodality imaging, these techniques play an indispensable role in the management of patients with benign PCTs by providing useful diagnostic and prognostic information to guide treatment. This article reviews the multimodality imaging characterizations of common benign PCTs.

A Case of Papillary Fibroelastoma of the Aortic Valve Causing an Embolic Ischemic Stroke.

Papillary fibroelastomas (PFEs) are the second most common primary cardiac tumors after myxomas. They are typically located on the aortic valve and comprise a short pedicle with multiple papillary fronds. PFEs are benign but highly friable in nature. Patients can be asymptomatic or present with severe thromboembolic complications. Echocardiography is the modality of choice for the diagnosis of these masses and surgical resection is indicated even in asymptomatic patients. Here, we have presented a case of a 53-year-old male who presented with a stroke after embolization of a PFE.

A Right Atrial Mass with Fever and Cutaneous Nodules.

Primary cardiac tumors, although exceedingly rare, should be considered in the differential diagnosis during workup of any cardiac mass. Extranodal cardiac lymphomas have a natural aggressive course due to delayed diagnosis. We present a 71-year-old male with a dual-chamber pacemaker who presented with fevers and new cutaneous nodules. He was found to have a right atrial primary anaplastic large-cell lymphoma and had a complete metabolic response after chemotherapy. Our case highlights the importance of a multimodality approach in the diagnosis of cardiac tumors and during follow-up after treatment.

Cardiac Papillary Fibroelastoma: Pulmonic Valve Involvement With Pulmonary Embolism and Pulmonary Hypertension.

Papillary fibroelastomas are rarely seen tumors of the cardiac valves. We present a case of papillary fibroelastoma present on the pulmonic valve. Diagnosis was made by characteristic echocardiogram findings. Complications of pulmonary embolism and pulmonary hypertension were present. Due to contraindications, the patient was managed with anticoagulation instead of surgery.

A Rare Case of Primary Cardiac Diffuse Large B-cell Lymphoma Imaged with 18F-FDG PET/CT.

Primary cardiac lymphoma is an extremely rare malignancy. A few reports about the findings of 18fluorine-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) imaging has been presented. We report a rare case of a 70-year-old male with diagnosed primary intracardiac diffuse large B-cell lymphoma referred for 18F-FDG PET/CT imaging for initial staging. The scan revealed an abnormal hypermetabolic gross tumoral lesion involving the right atrium and auricula. After completing three cycles of chemotherapy, post-treatment 18F-FDG PET/CT showed complete response.