Leiomyosarcomas (LMS) of the inferior vena cava (IVC) are a rare form of retroperitoneal malignancy, and their venous extension to the right atrium is an even rarer event. These tumors pose a unique surgical challenge and often require a multidisciplinary team-based approach for their surgical treatment. We present a case of a 68-year-old man with primary LMS of the IVC with a tumor thrombus extending into the right atrium that was initially deemed inoperable. After extensive neoadjuvant chemo-radiation with minimal tumor effect, the patient underwent en bloc surgical resection of the tumor along with removal of the infrarenal IVC and right kidney and adrenal without the need for cardiopulmonary bypass. This case demonstrates the successful management of a primary LMS of the IVC with right atrial extension using a multimodal approach of neoadjuvant chemo-radiation and en bloc surgical resection without cardiopulmonary bypass. This strategy may offer a curative option for selected patients with these rare and aggressive tumors, improving their survival and quality of life.

Despite its rare incidence of 1/40,000, fetal cardiac rhabdomyoma (CR) represents the prevailing type of benign cardiac fetal tumors, which commonly affects the ventricles. Fetal CRs rarely occur in the right atrium. Thus, the presentation of atrial fibrillation and premature atrial contractions (PAC) due to a solitary cardiac rhabdomyoma is an extremely rare scenario. Our literature review found that only 2% (1 out of 61) of rhabdomyoma cases were found in the right atrium. The majority of fetal cardiac rhabdomyomas are associated with tuberous sclerosis complex (TSC).
A 7-day-old male neonate presented with arrhythmias and an atrial mass for further evaluation. Echocardiography revealed a hyperechoic, round, uniform right atrial mass (25 mm). An abdominal and testicular ultrasound showed multiple thin-walled cortical cysts in both kidneys and a scrotal hydrocele, respectively. His laboratory workup was insignificant except for hypomagnesemia. Electrocardiography revealed junctional rhythm and PACs with wave distortions. A brain magnetic resonance imaging scan revealed multiple subependymal lesions on the frontal and occipital horns of the lateral ventricles. These findings (Fig. 1), along with a family history of TSC, confirmed the diagnosis of TSC with associated CR. The patient was treated symptomatically with an anti-convulsant and monitored with regular follow-ups. Surgical resection was not required.
Despite CR\'s predominance in the ventricles, a diagnosis of rhabdomyoma should be kept in mind in the presence of a solitary atrial mass and PACs. Physicians should evaluate systemic findings related to TSC and provide appropriate follow-up and family screening. Surgical resection is not always required, and symptom management can be achieved through medical treatment alone.

Invasive cardiac lipoma is a rare type of primary cardiac tumor that is composed of adipose tissue but infiltrating the adjacent structures. It is a benign tumor that can cause significant morbidity and mortality due to its size and location within the heart. We describe a giant invasive intracardiac lipoma across atrial wall extending to the ascending aorta and the superior vena cava. This review will provide an overview of invasive cardiac lipoma, including its clinical presentation, diagnosis, and management.

UNASSIGNED: Primary cardiac tumors are rare, and malignant primary cardiac tumors are even rarer. Cardiac osteosarcoma is a very rare type of malignant primary cardiac tumor with limited reported cases. We present a case report of cardiac osteosarcoma and review its characteristics and the related literature.
UNASSIGNED: A 44-year-old female patient without a specific medical history presented with intermittent dyspnea that started 1 month prior to presentation. A heterogeneous mass was observed in the left atrium on echocardiography and a large mass was observed in the left atrium on computed tomography. Surgery was performed under the suspicion of atypical cardiac myxoma, and the tumor was successfully removed. However, postoperative histopathological examination revealed cardiac osteosarcoma. The patient underwent chemotherapy and has been well maintained without recurrence for 10 years.
UNASSIGNED: We present a case report of the echocardiographic features and treatment strategies for cardiac osteosarcoma, an extremely rare cardiac tumor. Multimodal imaging can be helpful; however, a histological diagnosis through surgical resection is essential. Appropriate treatment and follow-up based on histological findings are necessary.

BACKGROUND: Cardiac myxoma is the most common type of primary cardiac tumor, with the majority located in the atrial wall. The tumor is attached to valvular structures in a few cases, of which the pulmonary valve is the least affected. Pulmonary valve myxoma may have different clinical manifestations from the more common cardiac myxomas because of its vital position. A misdiagnosis of these types of cardiac myxoma may be detrimental to the care and well-being of patients. Therefore, this systematic review aims to define the clinical characteristics of pulmonary valve myxoma and how this differs from a more common cardiac myxoma.
METHODS: Employed literature was obtained from PubMed, ScienceDirect, Scopus, Springer, and ProQuest without a publication year limit on August 23, 2022. The keyword was \"pulmonary valve myxoma.\" Inclusion criteria were as follows: (1) case report or series, (2) available individual patient data, and (3) myxoma that is attached to pulmonary valve structures with no evidence of metastasis. Non-English language or nonhuman subject studies were excluded. Johanna Briggs Institute checklists were used for the risk of bias assessment. Data are presented descriptively.
RESULTS: This review included 9 case reports from 2237 articles. All cases show a low risk of bias. Pulmonary valve myxoma is dominated by males (5:4), and the patient\'s median age is 57 years with a bimodal distribution in pediatric and geriatric populations. The clinical manifestation of pulmonary valve myxoma is often unspecified or asymptomatic. However, systolic murmur in the pulmonary valve area is heard in 67% of cases. Echocardiography remains the diagnostic modality of choice in the majority of cases. Tumor attached to the pulmonary cusps or annulus and extended to adjacent tissues in all cases. Therefore, valve replacement or adjacent tissue reconstructions are required in 77% of cases. The recurrence and mortality are considerably high, with 33% and 22% cases, respectively.
CONCLUSIONS: Pulmonary valve myxoma is more common in males with a bimodal age distribution, and its outcomes seem worse than usual cardiac myxomas. Increasing awareness of its clinical symptoms, early diagnosis, and complete myxoma resection before the presence of congestive heart failure symptoms are important in achieving excellent outcomes. A firm embolization blockade is needed to prevent myxoma recurrence.

BACKGROUND: The incidental detection of a right atrial mass during routine cardioncological workup is a rare condition. The correct differential diagnosis between cancer and thrombi is challenging. A biopsy may not be feasible while diagnostic techniques and tools may not be available.
METHODS: We report the case of a 59-year-old female patient with a history of breast cancer and current secondary metastatic pancreatic cancer. She developed deep vein thrombosis and pulmonary embolism and was admitted to the Outpatient Clinic of our Cardio-Oncology Unit for follow-up. Transthoracic echocardiogram incidentally found a right atrial mass. Clinical management was difficult due to the abrupt worsening of the patient\'s clinical condition and the progressive severe thrombocytopenia. We suspected a thrombus, according to its echocardiographic appearance, the patient\'s cancer history and recent venous thromboembolism. The patient was unable to adhere to low molecular weight heparin treatment. Due to worsening prognosis, palliative care was recommended. We also highlighted the distinguishing features between thrombi and tumors. We proposed a diagnostic flowchart to aid diagnostic decision making in the case of an incidental atrial mass.
CONCLUSIONS: This case report highlights the importance of cardioncological surveillance during anticancer treatments to detect cardiac masses.

In this report, we describe a 42-year-old man with dyspnea and recurrent hemoptysis who was diagnosed with cardiac angiosarcoma and multiple pulmonary metastases. Before visiting our hospital, he had been misdiagnosed with a lung infection. Bronchoscopy, multiple imaging methods, and extensive laboratory studies failed to identify a clear etiology. Cardiac magnetic resonance imaging and percutaneous lung biopsy revealed cardiac angiosarcoma with multiple pulmonary metastases. Cardiac angiosarcoma is extremely rare and diagnosis is difficult. Diffuse alveolar hemorrhage is not a common manifestation of pulmonary metastases of angiosarcoma and in patients with hemoptysis, clinicians should consider a malignant etiology that is not restricted to the lungs.

Cardiac lipomas are generally asymptomatic even in large dimensions. Echocardiograms can identify tumors, but cardiac magnetic resonance imaging or cardiac computerized tomography can differentiate cardiac lipomas from other cardiac tumors. The present study is a case report of an asymptomatic 30-year-old man diagnosed with atrial lipoma. The patient received cardiac surgery and the intervention consisted of exclusion of the right atrial (RA) tumor and reconstruction of the right atrium with \'XenoSure\' patch in extracorporeal circulation through a minimally invasive approach. A short PubMed literature review was performed and 26 cases of RA lipomas with available details were found. Cardiac tumors may cause clinical presentation through different pathways. Symptoms related to an RA lipoma were present in 21 out of 26 patients (80%). The symptoms varied greatly, dyspnea being the most common of them. In one case, the lipoma was found during the autopsy of a patient after sudden death. Large cardiac lipomas can lead to complications such as obstruction of ventricular outflow tract, electric disorders, embolism or pericardial effusion. Obstruction of the right ventricular outflow tract was reported in 11 out of 26 patients (42%) diagnosed with RA lipoma. Generally, atrial lipoma can have various sizes. The most useful imaging technique was transthoracic echocardiography. Accurate diagnosis and evaluation of cardiac lipoma is dependent on multimodality imaging methods, including cardiac magnetic resonance. Surgery is the treatment of choice, but the risk-benefit ratio must be considered, and shared decision making must be taken into account. The present review data showed that 23 out of 25 patients (92%) underwent surgery. Among these patients, only 1 out of 23 received a minimally invasive approach in 2021. Cardiac lipomas are rare entities, usually asymptomatic, that can occur at any age. The most useful diagnostic method of cardiac tumors is echocardiography, but nuclear magnetic resonance can also specify the type and characteristics of tumors.

Solitary fibrous tumor (SFT) are rare spindle cell tumors originating from the mesenchymal cells mostly from the visceral pleura. SFT was first described as a distinct entity in 1931 by Klemperer et al. Until now, we have limited data regarding the manifestation and behavior of extra pleural forms such as cardiac SFT. Here we present a case of SFT involving the pericardium where the diagnosis was made by imaging followed by biopsy findings. We also review the literature of SFT involving the heart and the management approaches.
An 81-year-old male presented with progressive dyspnea. Computed tomography (CT) of the chest showed a 6.2 × 5.3 cm soft tissue mass in the anterior mediastinum. Further imaging with CT angiogram showed a stalk-like connection to the pericardium. A biopsy of the mass showed spindle cells positive for BCL-2, CD34, and STAT 6, indicative of a solitary fibrous tumor. A surveillance approach was adopted for the patient.
Primary pericardial tumors are exceedingly rare, with a prevalence rate of 0.001%-0.007%. Diagnosing a SFT requires a positive CD34 and BCL-2 marker. The current recommendation is resection of localized disease which has been documented to be curative in cases of benign disease however our patient was put on surveillance.

BACKGROUND: Cardiac angiosarcoma is a very rare disease. As a result of their nonspecific presentation symptoms, and the lack of consensus in treatment, caution should be taken in both diagnosis and treatment. The role of radiotherapy (RT) is debatable due to the continuous movement of the heart, which makes it difficult to safely deliver high radiation doses to the target volume.
METHODS: The case of a 16-year-old boy with cardiac angiosarcoma that recurred one year after surgery and was treated with chemotherapy is presented. The patient received high field 1.5-Tesla (magnetic resonance) MR-Linac treatment in 5 fractions with a dosage of 25 Gy to the tumor bed and 30 Gy to the recurrent nodules using the simultaneous integrated boost technique. The patient tolerated the treatment well and had stable disease two months later.
CONCLUSIONS: MR-guided radiotherapy, particularly in the case of cardiac malignancies, allows for direct tumor visualization with high soft tissue image resolution capacity. Furthermore, modern RT techniques allow for the full therapeutic window to be used by achieving superior dose distributions, allowing for dose escalation strategies with tolerable toxicity rates.
CONCLUSIONS: Magnetic resonance guided RT allows direct visualization of the target during treatment delivery, allowing for higher-dose administration with less damage to healthy tissue near the tumor. This treatment strategy is a viable option in selected patients with cardiac angiosarcoma.