Overall approaches to cardiac tumors: Still an unsolved enigma?

Cardiac tumors are neoplasms involving heart structures at any level, meaning the myocardium, valves, and cardiac chambers. When considering cardiac masses, it is not uncommon for surgeons to be surprised when they diagnose one. The real incidence of this complex group of diseases has been explored only after cardiac diagnostic tools became more appropriate. Despite differential diagnosis being relevant, surgical indication is usually requested for all malignant cardiac tumors and also for many types of benign tumors. The development of cardiac imaging techniques, therefore, has been the key point for a better understanding of the history of cardiac tumors and especially of the relevance of surgical indication in such conditions. Systematic and combined applications of echocardiography, cardiac computed tomography and magnetic resonance allow in the majority of case a clear definition of the nature of a newly discovered cardiac mass. The presence of a Li-Fraumeni syndrome seems to be the trigger aspect in accelerating the propensity of developing a cardiac tumor. Despite the revolutionary usefulness of the cardiac imaging techniques available, it is still considered a hazard to diagnose a malignant cardiac mass just with radiological imaging; the mainstay of the final diagnosis stands in surgical excision of the mass and histopathological report.

Calcified amorphous tumor attached to the membranous septum: a rare clinical entity.

We report a rare case of a pedunculated calcified amorphous tumor (CAT) of the left ventricle attached by a stalk to the membranous septum in a 74-year-old woman who presented with a cerebrovascular accident. We believe this is the first report of a CAT attached to the membranous septum.

Association between gut microbiota and malignant cardiac tumors: A two-sample Mendelian randomization study.

BACKGROUND: Recent studies provide compelling evidence linking the gut microbiota to most cancers. Nevertheless, further research is required to establish a definitive causal relationship between the gut microbiota and malignant cardiac tumors. METHODS: The genome-wide association studies (GWAS) data on the human gut Microbiota, included in the IEU Open GWAS project, was initially collected by the MiBioGen consortium. It encompasses 14,306 individuals and comprises a total of 5,665,279 SNPs. Similarly, the GWAS data on malignant cardiac tumors, also sourced from the IEU Open GWAS project, was initially stored in the finnGen database, including 16,380,303 SNPs observed within a cohort of 174,108 individuals within the European population. Utilizing a two-sample Mendelian randomization (MR) methodology, we examined whether there exists a causal association between the gut microbiota and cardiac tumors. Additionally, to bolster the credibility and robustness of the identified causal relationships, we conducted an extensive array of sensitivity analyses, encompassing Cochran's Q test, MR-PRESSO tests, MR-Egger interpret test, directionality test and leave-one-out analysis. RESULTS: Our analysis unveiled seven distinct causal associations between genetic susceptibility in the gut microbiota and the incidence of malignant cardiac tumors. Among these, the Family Rikenellaceae, genus Eubacterium brachy group, and genus Ruminococcaceae UCG009 exhibited an elevated risk of cardiac tumors, while the phylum Verrucomicrobia, genus Lactobacillus, genus Ruminiclostridium5, and an unknown genus id.1868 were genetically linked to a reduced risk of cardiac tumors. The causal relationship between these two bacteria, belonging to the phylum Verrucomicrobia (OR = 0.178, 95% CI: 0.052-0.614, p = 0.006) and the genus Ruminococcaceae UCG009 (OR = 3.071, 95% CI: 1.236-7.627, p = 0.016), and cardiac tumors was further validated through sensitivity analyses, reinforcing the robustness and reliability of the observed associations. CONCLUSION: Our MR analysis confirms that the phylum Verrucomicrobia displays significant protection against cardiac tumor, and the genus Ruminococcaceae UCG009 leads to an increasing risk of cardiac tumor.

Cardiac Angiosarcoma Presenting as Large Pericardial Effusion With Effusive-Constrictive Pericarditis.

Pericardial angiosarcoma is an extremely rare malignant tumor originating from the endothelial cells of blood vessels within the pericardium. We present a case of a 49-year-old male who presented with symptoms of pericardial effusion and was subsequently diagnosed with pericardial angiosarcoma. This case report highlights the diagnostic challenges and management options associated with this rare entity.

Case Report: Assessment of primary myxofibrosarcoma in the left atrium using multimodal ultrasonography.

Primary myxofibrosarcoma of the heart, a rare cardiac malignancy, was diagnosed in a middle-aged female patient exhibiting progressive dyspnea following transthoracic echocardiography and pathological analysis. Postoperatively, the patient underwent chemotherapy and Lenvatinib mesylate therapy, with regular check-ups confirming her survival. After 10 months the patient is still alive and well.

A case of surgical treatment for recurrence of right ventricular metastasis due to renal cell carcinoma after molecular targeted therapy.

BACKGROUND: Cardiac metastasis including the right ventricle from renal cell carcinoma is rare. No standard treatment for cardiac metastasis and recurrence in renal cell carcinoma has been established. CASE PRESENTATION: We present the case of a 61-year-old man who underwent the resection of recurrent right ventricular metastasis caused by renal cell carcinoma following molecular targeted therapy. The first cardiac operation was performed for right ventricular metastasis due to renal cell carcinoma. The patient had a good postoperative course. Two years after the first operation, however, follow-up computed tomography revealed the recurrence of the right ventricular tumor and metastases in both lungs. Molecular targeted therapy was carried out and effectively controlled the lung metastasis but the right ventricular lesion remained unchanged, leading to reoperation. The recurrent right ventricular tumor was completely resected through a redo median sternotomy assisted by cardiopulmonary bypass. The patient had an uneventful postoperative course and was discharged on the 13th postoperative day. Follow-ups at 2 years showed no cardiac recurrence. CONCLUSION: Surgical intervention was considered useful in managing the recurrence of right ventricular metastasis from renal cell carcinoma after molecular targeted therapy.

Glandular cardiac myxoma with intraepithelial ductal neoplasia features: Report of the first case.

Cardiac myxoma are the most common primary tumor of the heart in adults. In approximately 2-5%, glandular differentiation occurs within these tumors. In the presence of glandular features attention must be taken to exclude and prevent a misdiagnosis of cardiac metastases of adenocarcinoma. Nevertheless, the localization in the left atrium, the solitary disposition of the cardiac mass, the histological features and the immunohistochemistry performed, argued against the possibility of a metastatic nature of the tumor. We report the case of an 80-year-old woman, with a prior medical history of breast cancer, that underwent surgery for a cardiac myxoma that histologically showed glandular features. Herein, we highlight the importance of a careful diagnosis of this entity, as it can be easily confused for a metastasis, especially in patients with a history of malignancy.

Yearlong Near-Syncope: Presentation of a Rare Right Atrial Myxoma.

Atrial myxomas are the most common form of primary benign cardiac tumors. The left atrium is typically the most common location while right atrial myxomas are much rarer and only occur in about 15%-25% of all myxoma patients. Typically, left atrial myxomas have the ability to cause symptoms such as syncope. We report a case of a 67-year-old female who presented with complaints of palpitations, dizziness, and near-syncope that had been ongoing for about a year. Other causes of syncope were investigated and ruled out. A transthoracic echocardiogram (TTE) found a large 4.3 x 4.0 cm spherical mass in the right atrium which was confirmed by surgical resection and immunohistochemistry to be a myxoma. The patient's condition of syncope-like symptoms warrants elevating atrial myxomas to a higher position in the diagnostic differential.

Right ventricular outflow tract obstruction by cardiac hemangioma in asymptomatic patient.

Ventricular hemangiomas are rare benign tumors, pose diagnostic and therapeutic complexities. We report a case of a 52-year-old female with essential hypertension who developed a systolic ejection murmur during a hypertension clinic visit. The echocardiogram revealed a hyperechoic mass obstructing the right ventricular outflow tract, causing enlargement of the right atrium and ventricle, with a reduction in the right ventricular ejection fraction. Due to the risk of death, the patient underwent an emergency surgical resection along with tricuspid valve replacement. Postoperative recovery was uneventful, and subsequent cardiac magnetic resonance imaging showed an improvement in ejection fraction without residual tumor. This case highlights the diagnosis and therapeutic complexities of ventricular hemangiomas. With this report, we aim to provide a comprehensive review of ventricular hemangiomas and to enhance understanding of this condition for improved patient care.

Incidental cardiac lymphangioma misdiagnosed as atrial thrombus: A case report.

Cardiac lymphangioma is a characteristically benign primary neoplasm of the heart, previously reported only in a handful of cases. A right atrial lesion was found of a 56-years old healthy male patient. The lesion was surgically excised and identified as cardiac lymphangioma in postoperative pathological analysis. While open surgical tumor resection is preferred in patients with cardiac lymphangioma, preoperative characterization of suspected lesions may warrant conservative management in selected cases.

Treatment of Primary Cardiac Tumors in Children: A Single Center 18-Year Experience.

This study aimed to compare the clinical characteristics and courses of pediatric patients with cardiac tumors in nonoperative and operative groups to help guide treatment decisions. We reviewed the medical records of patients diagnosed with primary pediatric cardiac tumors at our institution between 2003 and 2020. Demographic data, clinical characteristics, and follow-up data between the operation and nonoperation groups were compared. A total of 56 patients were included in the study. Thirteen patients underwent surgery. The median age was 1.4 months (range, 1 to 18 years). The patients in the operation group had more frequent symptoms or signs, such as desaturation, respiratory difficulty, murmur, a higher mass area/chamber area (MC) ratio, decreased ventricular contractility, and significant ventricular outflow tract obstruction (VOTO). An MC ratio of 0.568 was the cutoff value for differentiating patients with symptoms or signs of heart failure and decreased ventricular contractility. At the last follow-up, all patients had good ventricular contractility except one patient in the operative group with fibroma. In the non-operative group, rhabdomyomas often regressed spontaneously, while fibromas often increased in size. Two patients in the nonoperative group died. In the operative group, there was no early or late mortality or tumor recurrence. In this study, patients had good outcomes with or without surgery, even when the tumor was large, or surgery was performed in early infancy.

A case of cardiac undifferentiated pleomorphic sarcoma in late pregnancy: A case report.

Cardiac tumors are uncommon and most of them are benign. Although cases of malignant cardiac tumors are rare, it is still necessary to improve awareness in both clinical and pathological diagnosis. Since cardiac tumors often have a high degree of malignancy, it is vital to determine what form of intervention can increase recurrence-free survival and overall survival. In this paper, we report on a 42-year-old woman in the third trimester of pregnancy who had a cardiac undifferentiated pleomorphic sarcoma. According to her medical history, the patient had never had a cardiac tumor or any other disease. She was treated surgically and a left atrial mass was removed immediately after cesarean section. No other treatments were applied after the surgery, and, unfortunately, the tumor reoccurred 6 months later. We reviewed some literature and found one case in which the patient was treated using radiotherapy and survived for another 2 years after the third tumor recurrence. This suggests that neoadjuvant therapy effectively improves the survival rates of such patients.

Unusual Location of a Papillary Fibroelastoma Causing a Multi-territorial Stroke.

A 56-year-old male presented with a multi-territorial stroke without traditional cerebrovascular risk factors. A transesophageal echocardiogram revealed an intracardiac lesion attached to the lateral wall of the left atria, consistent with an atrial myxoma. Surgical excision of the lesion was performed and revealed that lesion was in fact a papillary fibroelastoma with thrombus attached, which demonstrates a novel mechanism by which intracardiac masses can cause cerebral events.

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.

Biatrial myxoma in a young lady.

A healthy 28-year-old woman, presenting with a chronic cough for approximately 6 months, was referred for echocardiography. The images revealed the presence of two masses in each atrium without an inter-atrial septal defect. No additional abnormalities were detected during the clinical examinations. Subsequently, the patient underwent a successful surgical procedure for the removal of the cardiac masses.

Complete resection of a giant intrapericardial cardiac synovial sarcoma.

Synovial sarcoma of the heart is a rare tumor. Herein we would like to report a case of giant intrapericardial cardiac synovial sarcoma that originated from the right ventricle and grew outward near the diaphragm. After making adequate preoperative preparation, we performed the surgery as quickly as possible and resected the tumor completely. Based on the identification of the translocation on chromosome 18 rearrangement, the tumor can be diagnosed as a primary cardiac synovial sarcoma. Through this study, we aim to afford more information about cardiac synovial sarcomas as well as a reference for similar cases.

Case report: Characteristics and nature of primary cardiac synovial sarcoma.

Primary malignant cardiac tumors rarely occur, and cardiac synovial sarcoma (SS) is especially rare among such tumors. Herein, we present the case of a 35-year-old female with primary cardiac SS treated with surgery, chemotherapy, and radiotherapy. She presented with chest symptoms and underwent imaging examinations. A cardiac tumor was suspected, and an open biopsy was performed. The pathological findings suggested cardiac SS. Next, we performed a resection, and the tumors persisted at a macroscopic level. Immunohistochemistry was negative for SS18-SSX and positive for the SSX C-terminus and cytokeratin CAM5.2, a reduction of SMARCB1/INI1 was observed, and fluorescence in situ hybridization showed positive SS18 split staining. Owing to the FNCLCC grade 3 tumor and R2 margins, adjuvant chemotherapy with ifosfamide, doxorubicin, and radiotherapy was initiated, and the patient was diagnosed with cardiac SS. The differences in patients with cardiac SS compared with general SS include male predominance, larger tumor size, and poorer prognosis. Pathological findings of immunohistochemistry and fluorescence in situ hybridization were found to be more reliable than imaging findings for a correct diagnosis. Additionally, because incomplete resection is frequently performed, adjuvant therapy, including chemotherapy and radiation therapy, may be performed. The findings indicate that multiple therapies, including surgery, chemotherapy, and radiotherapy, are essential treatment strategies for improving the prognosis of patients with cardiac SS.

Arrhythmogenic Left Ventricular Mass as the Initial Presentation of Cardiac Sarcoidosis.

Cardiac tumors of the left ventricle are rare, and cardiac magnetic resonance is the preferred imaging tool for evaluation given superior tissue characterization. We present a case of a patient with arrhythmia and left ventricular mass that was ultimately diagnosed with cardiac sarcoidosis, reminding us that tissue is the issue.

Unusual presentation and delayed diagnosis of cardiac angiosarcoma.

BACKGROUND: Primary cardiac angiosarcomas are very rare and present aggressively with high rates of metastasis. Given the poor prognosis, particularly once disease has spread, early diagnosis and multidisciplinary treatment is essential. CASE PRESENTATION: We present the case of a 46-year-old male who presented with chest pain, intermittent fevers, and dyspnea. Workup with computed tomography scan and transesophageal echocardiography demonstrated a right atrial pseudoaneurysm. Given the concern for rupture, the patient was taken to the operating room, where resection of the pseudoaneurysm and repair using a bovine pericardial patch was performed. Histopathology report initially demonstrated perivascular lymphocyte infiltrate. Six weeks later, the patient represented with chest pain and new word finding difficulty. Workup revealed multiple solid lung, pericardial, brain, and bone nodules. Eventual biopsy of a cardiophrenic nodule demonstrated angiosarcoma, and rereview of the original pathology slides confirmed the diagnosis of primary cardiac angiosarcoma. CONCLUSIONS: Primary cardiac angiosarcomas are often misdiagnosed given the rarity of these tumors, but early diagnosis and initiation of treatment is essential. The unique presentation of our case demonstrates that clinical suspicion for cardiac angiosarcoma should be maintained for spontaneous pseudoaneurysm originating from the right atrium.

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.