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.

Cardiac angiosarcoma with pulmonary and adrenal metastases diagnosed by FDG-PET/CT-guided adrenal biopsy.

Primary cardiac angiosarcoma is a high-grade aggressive tumor with a poor prognosis and low incidence. We describe a case of cardiac angiosarcoma, with pulmonary and adrenal metastases, diagnosed via fluorodeoxyglucose-positron emission tomography/computed tomography-guided adrenal biopsy. Learning objective: Cardiac angiosarcoma should be considered in a patient with a cardiac mass with no tumor cells in the pericardial fluid. Fluorodeoxyglucose-positron emission tomography/computed tomography could be useful in determining the biopsy site.

Recurrent Pericardial Effusion Secondary to Cardiac Angiosarcoma.

Cardiac angiosarcoma is a malignant cardiac tumour. We present the case of a young patient in his mid-30s with recurrent pericardial effusion. He had flu-like symptoms a month earlier and had shortness of breath, lethargy, and tightness in his throat for the past ten days. Echocardiography demonstrated global pericardial effusion > 4 cm with tamponade features, and the patient was blue-lighted to our hospital. He underwent emergency pericardiocentesis, and > 1 litre of pericardial fluid was drained. Computed tomography of the chest, abdomen, and pelvis revealed small-volume ascites and moderate right-sided pleural effusion, with associated lobar collapse. The patient presented to the hospital with global pericardial effusion requiring emergency pericardiocentesis three weeks later and underwent cardiac magnetic resonance imaging demonstrating global pericardial effusion and a 48 × 26 mm pericardial space mass adjacent to the right atrium. He underwent surgical resection of the tumour, followed by chemotherapy, and tolerated the treatment well. The patient is currently under follow-up.

Unusual Finding in a Patient Presenting with Abdominal Pain and Dyspnea.

This article discusses the rarity of primary cardiac tumors, particularly angiosarcomas, accounting for 10% of primary cardiac neoplasms. The article highlights the challenge of early diagnosis due to nonspecific symptoms. The importance of sectional imaging, particularly computed tomography (CT) scans, in emergent situations is emphasized. Teaching point: Despite the rarity of primary cardiac tumors, radiologists should remain alert, especially when pericardial effusion is present.

Case Report: Two cases of advanced primary cardiac angiosarcoma treated with anlotinib and a retrospective analysis of the literature.

Primary cardiac angiosarcoma is a rare malignant soft-tissue sarcoma derived from vascular endothelial cells or lymphatic endothelial cells, with a high malignancy, poor prognosis, and a lack of effective medical therapy. This article reports on two patients with primary cardiac angiosarcoma who received first-line treatment with multi-targeted anti-angiogenic agent, anlotinib monotherapy. The treatment rapidly controlled pleural and pericardial effusion, significantly reduced the tumor, improved symptoms, and showed satisfactory recent efficacy. This indicates that anlotinib offers a new first-line treatment option for advanced primary cardiac angiosarcoma.

Radiotherapy combined with anti-PD-1 and TKI for primary cardiac angiosarcoma considering the joint assessment of TLSs and PD-L1: a case report.

BACKGROUND: Primary cardiac angiosarcoma(PCA) has a low incidence rate and poor prognosis. Currently, no unified clinical treatment standards are available. CASE PRESENTATION: We report the case of a 48-year-old man presenting chest tightness, breathlessness, and dyspnea. Imaging and postoperative histopathologic studies confirmed PCA and that the tumor had invaded the entire right atrium. The patient developed progressive disease (PD) during postoperative radiotherapy. We used immunotherapy combined with targeted therapy based on the results of molecular profile and evaluation of tertiary lymphoid structures (TLSs) and programmed cell death-ligand 1 (PD-L1). After treatment, the metastatic lymph nodes of the patient were reduced to a certain extent, indicating that combination therapy was effective. CONCLUSION: To the best of our knowledge, this is the first report of radiotherapy combined with anti-PD-1 and tyrosine kinase inhibitors(TKI) for PCA. In addition, this is the first report on immunotherapy for PCA based on new evaluation methods, including TLSs, PD-L1, and genomic profile.

A Giant Primary Angiosarcoma Invading the Right Heart in a Young Male: An Emergency Surgery.

Primary cardiac angiosarcoma is very rare. In this report, we describe an interesting case of a 25-year-old male with a giant primary angiosarcoma invading the right heart. He was urgently admitted to the hospital for respiratory distress. Once the diagnosis was suspected by chest x-ray, echocardiography, and CT scan, and given the patient's hemodynamic and respiratory instability, an emergency open-heart surgery was necessary to prevent complications. Through a right atriotomy and a pulmonary infundibulotomy, the tumor was resected. Invaded by the tumoral process, the tricuspid valve was replaced with a biological prosthesis. The postoperative course was marked by severe right ventricular dysfunction with multiorgan failure. Histopathologic examination of the surgical specimen confirmed a primary cardiac angiosarcoma.

Primary cardiac angiosarcoma: Imaging characterization.

This case report describes a 35-year-old female patient who presented with palpitations and shortness of breath. Imaging findings suggested a cardiac tumor, histopathology confirmed primary cardiac angiosarcoma. This tumor is highly aggressive, usually occurs in the right atrium, lacks specificity in clinical presentation, is prone to early metastasis, and has a poor prognosis. Echocardiography is the method of choice for early detection and is important in assessing tumor size, location, mode of attachment and whether cardiac function is impaired.

Case Report: Successful surgical management of a challenging primary cardiac angiosarcoma.

Primary cardiac tumors are exceptionally rare, with malignant tumor occurrences ranging from 0.0017% to 0.28%. Among these, primary cardiac angiosarcoma (PCA) stands as the most prevalent malignancy, primarily impacting the right cardiac system. In this case report, we present the instance of a 44-year-old woman who recently exhibited acute chest discomfort and was subsequently diagnosed with a microangiosarcoma within the right atrium and superior vena cava. Diagnostic modalities including chest x-rays, CT, MRI, and PET-CT were instrumental in pinpointing the tumor's location and nature. Surgical excision followed by pathological and immunological examinations confirmed the diagnosis. The patient's recovery post-surgery has been encouraging, with successful follow-up chemoradiotherapy administered. Despite advancements, devising optimal strategies for enhancing patient survival and quality of life in angiosarcoma cases remains a pressing research challenge.

Primary Cardiac Epithelioid Angiosarcoma with Sustained Remission following Surgical Resection: A Case Report.

Epithelioid angiosarcoma is an extremely rare subtype of cardiac angiosarcoma that is highly aggressive and associated with poor prognosis. Due to its rare nature, the epidemiology and pathogenesis of this disease are not well-known. Thus, effective diagnostic and treatment modalities are limited. Here, we report a case of a primary epithelioid angiosarcoma in a patient who was treated successfully with surgical resection. A 45-year-old woman who initially presented with chronic systemic symptoms and severe anemia and subsequently developed new-onset atrial fibrillation with rapid ventricular rate was found to have a right-sided cardiac mass with a large pericardial effusion. Several years prior to presentation, she was treated for localized papillary thyroid cancer with Gc protein-derived macrophage-activating factor (Gc-MAF) therapy after declining thyroidectomy. After initial workup of her cardiac mass, including a transthoracic echocardiogram and cardiac MRI, she was transferred to an outside hospital where her mass was surgically resected. She was found to have stage IIIA high-grade epithelioid angiosarcoma involving the inferior vena cava, right atrium, and pericardium. She subsequently had complete resolution of her pericardial effusion and anemia and continues to have good performance status 16 months after her excellent surgical outcome without evidence of recurrence. This unique case contributes to our knowledge of epithelioid cardiac angiosarcoma, of which limited number of cases has been reported. It highlights a favorable outcome following surgical resection of a rare, life-threatening primary cardiac tumor.

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.

Multimodality treatment of primary cardiac angiosarcoma: A systematic literature review.

BACKGROUND: Primary cardiac angiosarcoma (PCA) is the most prevalent histological type of cardiac sarcoma but its rarity poses a challenge for standardizing treatment protocols. Moreover, published studies are limited by small patient numbers and lack of randomization, making it challenging to establish evidence-based treatment strategies. This systematic review aims to consolidate the heterogeneous published data and identify factors related to the treatment outcome of PCA patients. METHODS: The PubMed and Scopus bibliographic databases were systematically searched for original articles reporting clinical, treatment and outcome data for PCA patients. Kaplan-Meier analysis was used to calculate the time to progression and survival. The Log-Rank test was used to compare progression-free and overall survival data. The Cox proportional hazards regression model was used for univariate and multivariate analysis of survival data. RESULTS: A total of 127 studies containing data for 162 patients were analyzed. The median age of the patient cohort was 45 years, with males being 1.5 times more frequently affected than females. Tumors were primarily located on the right side of the heart, with a median size of 6 cm. Median progression-free and overall survival of 5 months and 12 months, respectively, were calculated. Age, sex, and resection margins did not have a significant impact on PCA survival, as determined by both univariate and multivariate analyses. The presence of metastases at diagnosis was associated with lower overall survival in univariate analysis, although this effect was not significant in multivariate analysis. Multimodality treatment that incorporated surgery and adjuvant chemo-radiotherapy was associated with a statistically significant survival benefit. Median overall survival increased from 6 months with surgery alone to 13 months and 27 months with adjuvant chemotherapy and chemo-radiotherapy, respectively. CONCLUSION: Multimodality treatment including surgery and chemo-radiotherapy was found to offer the greatest survival benefit for PCA patients.

Primary Cardiac Angiosarcoma: A Review.

Primary cardiac angiosarcoma is a rare and aggressive malignancy originating from the endothelial lining of cardiac blood vessels. This review covers various aspects of the disease, including its pathogenesis, clinical presentation, diagnosis, treatment, and prognosis. The primary characteristic of cardiac angiosarcoma is the rapid growth of abnormal blood vessels that invade the heart muscle, leading to the destruction of healthy tissue. Due to its infiltrative nature and early spread, diagnosing and treating cardiac angiosarcoma present significant challenges. Transesophageal echocardiography (TEE) plays a crucial role in diagnosing cardiac tumors such as angiosarcoma due to its high sensitivity. Additional imaging techniques such as computed tomography (CT) and cardiac magnetic resonance imaging (MRI) help assess tumor anatomy and identify metastases. Histopathological examination and immunohistochemistry are essential for confirming the diagnosis, as they reveal distinct histological features and specific endothelial markers associated with primary cardiac angiosarcoma. Targeted therapies directed at the angiogenic mechanisms and molecular abnormalities hold promise for improving treatment outcomes. Early detection of primary cardiac angiosarcoma remains challenging due to its rarity, and the prognosis is generally poor due to advanced disease at the time of diagnosis. The review emphasizes the importance of a multidisciplinary approach and collaboration among different specialties to optimize the diagnosis, treatment, and follow-up care of patients with primary cardiac angiosarcoma. The ultimate goal is to enhance diagnostic methods and therapeutic approaches by advancing knowledge and promoting further research into this aggressive malignancy.

Primary cardiac tumor: a case report of right atrial angiosarcoma and review of the literature.

Primary cardiac angiosarcoma is a relatively rare tumor with early metastasis and poor prognosis. Radical resection of the primary tumor remains the primary approach for the optimal survival of patients with early-stage cardiac angiosarcoma without evidence of metastasis. This case involves a 76-year-old man with symptoms of chest tightness, fatigue, pericardial effusion, and arrhythmias who achieved good results after surgery to treat the angiosarcoma in the right atrium. In addition, literature analysis showed that surgery remains an effective way of treating primary early angiosarcoma.

Primary Cardiac Sarcoma: Angiosarcoma Compressing the Right Coronary Artery.

Cardiac tumors are uncommon and can be classified as either primary benign, primary malignant, or metastatic. Cardiac tumors have a wide range of presentations, which can lead to delays in diagnosis and treatment. Primary cardiac tumors can also affect nearby structures, and there have been a few reported cases of coronary artery involvement with various underlying causes. In this case report, we describe a patient with a primary cardiac sarcoma (angiosarcoma) that had spread to other parts of the body and caused occlusion of the right coronary artery.

Primary cardiac angiosarcoma initially diagnosed on pericardial fluid cytology with histology and autopsy correlation.

Primary cardiac angiosarcoma is an exceedingly rare high-grade malignancy of the heart originating from endothelial cells, with a predilection for the right atrium in male. Clinical diagnosis is extremely challenging because of the nonspecific symptoms and radiological findings. Although almost always presenting with massive recurrent pericardial effusions, cardiac angiosarcoma diagnosed based on pericardial fluid has rarely been reported, either due to the paucity of malignant cells or misdiagnosis due to low familiarity/suspicion and lack of proper workup. Unfortunately, patients with this disease often receive definitive diagnosis post-mortem. We report a case of primary cardiac angiosarcoma initially diagnosed on pericardial fluid. The cytomorphology and immunophenotype of angiosarcoma in fluid, as well as the challenges and practical recommendations in using pericardial fluid cytology for early diagnosis of this deadly disease are discussed.

Multimodal imaging of primary cardiac angiosarcoma with brain metastasis.

Angiosarcoma is an extremely rare primary cardiac malignant tumor, with characteristics of early blood metastasis and radiochemotherapy resistance. Early diagnosis and timely treatment are of great significance to the prognosis of patients. Hereinafter, we report a case of angiosarcoma in the left atrium of a 61-year-old woman who underwent multimodality imaging and successful resection of the angiosarcoma. Results of the present case suggest that multimodal imaging plays an important role in detecting angiosarcoma and determining the treatment plan and prognosis for patients after treatment.

Treatment of an unresectable cardiac angiosarcoma using paclitaxel and image guided radiotherapy: Case report and literature review.

Primary cardiac angiosarcomas (PCAs) are rare tumors that are typically found in the right atrium between the third and the fifth decade of life. While surgical removal of the tumor combined with adjuvant chemotherapy and/or radiotherapy is the treatment of choice, most of the patients present with unresectable tumors and metastatic disease carrying a dismal prognosis with a median survival of less than 1 year. Doxorubicin and ifosfamide based chemotherapy combined with radiotherapy is currently employed in these patients, but no standardized treatment algorithms exist. In this report, we present the management of a patient with an unresectable PCA treated using weekly paclitaxel (120 mg) combined with radiotherapy (60 Gy in 30 fractions) delivered by a helical TomoTherapy system. Follow-up imaging studies showed a remarkable tumor regression which allowed for surgical excision of the tumor 10 months post treatment. Histopathological examination of the resected mass showed no viable tumor cell. On the latest follow-up study, 12 months post treatment, no sign of disease progression (local or distant) was found, and the patient is in good clinical condition.