Cardiac Angiosarcomas Research Articles

Primary Cardiac Angiosarcoma with Extensive Visceral Metastases: Utility of 18F-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography in Response Assessment to Sorafenib.

Primary cardiac angiosarcomas although rare neoplasms remain the most common primary malignant neoplasms affecting the heart. We discuss the sequential positron-emission tomography–computed tomography findings of a 50-year-old man who was diagnosed with the metastatic cardiac angiosarcoma at the outset and developed progressive disease despite sorafenib therapy.

The wide spectrum of POT1 gene variants correlates with multiple cancer types.

The POT1 protein binds and protects telomeres. Germline variants in the POT1 gene have recently been shown to be associated with risk of developing tumors in different tissues such as familial chronic lymphocytic leukemia, colorectal, glioma and melanoma tumors. Recently, we uncovered a variant in the POT1 gene (p.R117C) as causative of familial cardiac angiosarcomas (CAS) in Li-Fraumeni-like (LFL) syndrome families. Our in silico studies predicted that this protein had lost the ability to interact with TPP1 and single-stranded DNA. In vitro studies corroborated this prediction and showed that this lack of function leads to abnormally long telomeres. To better understand the POT1 gene and its role with tumorigenesis, we extended the study to LFL (with and without members affected with angiosarcomas (AS)) and sporadic AS and cardiac sarcomas. We found POT1 variants in the 20% of the families with members affected with AS and 10% of sporadic AS and sarcomas. In silico studies predicted that these new variants were damaging in the same manner as previously described for the POT1 p.R117C variants. The wide spectrum of variants in the POT1 gene leading to tumorigenesis in different tissues demonstrates its general importance. Study of the POT1 gene should be considered as routine diagnostic in these cancers.

Case report: whole exome sequencing of primary cardiac angiosarcoma highlights potential for targeted therapies

BackgroundPrimary cardiac angiosarcomas are rare, but they are the most aggressive type of primary cardiac neoplasms. When patients do present, it is with advanced pulmonary and/or cardiac symptoms. Therefore, many times the correct diagnosis is not made at the time of initial presentation. These patients have metastatic disease and the vast majority of these patients die within a few months after diagnosis. Currently the treatment choices are limited and there are no targeted therapies available.Case presentationA 56-year-old male presented with shortness of breath, night sweats, and productive cough for a month. Workup revealed pericardial effusion and multiple bilateral pulmonary nodules suspicious for metastatic disease. Transthoracic echocardiogram showed a large pericardial effusion and a large mass in the base of the right atrium. Results of biopsy of bilateral lung nodules established a diagnosis of primary cardiac angiosarcoma. Aggressive pulmonary disease caused rapid deterioration; the patient went on hospice and subsequently died. Whole exome sequencing of the patient’s postmortem tumor revealed a novel KDR (G681R) mutation, and focal high-level amplification at chromosome 1q encompassing MDM4, a negative regulator of TP53.ConclusionMutations in KDR have been reported previously in angiosarcomas. Previous studies also demonstrated that KDR mutants with constitutive KDR activation could be inhibited with specific KDR inhibitors in vitro. Thus, patients harboring activating KDR mutations could be candidates for treatment with KDR-specific inhibitors.

Primary Right Atrial Angiosarcoma

Case: A 25-year-old male presented with 3 months of progressive dyspnoea. CT finding a RA mass and large pericardial effusion that was drained, cytology did not identify malignant cells. Subsequent cardiac MRI revealed a heterogeneous RA mass measuring 7 × 7 × 9 cm invading into the pericardium and suspended into the TV annulus. Transvenous biopsy under TOE and fluoroscopic guidance revealed angiosarcoma. The tumour could not be ressected due to the patient's body habitus (BMI 59) which prevented the use of CPB. The patient was referred for palliative chemoradiotherapy. Background: Primary cardiac tumors are rare, with an incidence at autopsy from 0.0017-0.033%. Prognosis is dismal with median survival reported at 25 months. Primary cardiac angiosarcomas (PCAs) have a tendency to occur in the third to fifth decade and are more common in males. 90% of angiosarcomas are located in the right atrium. The lateral (free) wall of the right atrium is the most common site, the septum being spared in most cases. The clinical presentation of PCAs is often non-specific. The tumour has a propensity to involve the right side and so may present with right-sided heart failure, SVC obstruction, recurrent pericardial effusions or cardiac tamponade. In rare cases, the tumour may present with haemopericardium from rupture. The primary goal of treatment is total resection with RA reconstruction. Partial resection with adjuvant chemotherapy may be performed for palliative reasons. Prognosis remains poor with total resection shown to increase survival from 6 to 25 months after initial detection.

Stereotactic Body Radiotherapy with Cyberknife for Cardiac Malignancies

Radiobiological and technical considerations have traditionally limited the role of radiation therapy in the context of primary and secondary cardiac malignancies. Stereotactic body radiotherapy (SBRT) is a promising modality for the delivery of focused high-dose radiation with ablative potential to complex targets such as small, deep-seated, moving lesions, allowing also for re-irradiation. Between January 2013 and October 2013, 3 patients underwent SBRT for cardiac lesions: 2 patients had recurrent, previously irradiated cardiac angiosarcomas (PCA) and 1 patient had a cardiac metastasis from melanoma. They were treated with fiducial-guided robotic radiotherapy with CyberKnife. As for dose prescription, 24 Gy in 3 fractions (80% isodose) and 30 Gy in 5 fractions (80% isodose) were administered to the recurrent PCAs and cardiac metastasis, respectively. At 2 months after SBRT, cardiac MRI showed a partial response in the patients treated for recurrent PCA while the cardiac metastasis remained stable. In all cases, absence of local progression was subsequently confirmed by contrast-enhanced cardiac MRI after 6 months, without any evidence of treatment-related side effects. Fiducial-guided SBRT proved to be feasible and effective in preventing local disease progression in selected patients with cardiac malignancies.

18F-FDG PET-CT for Evaluation of Cardiac Angiosarcoma: A Case Report and Review of Literature.

Cardiac angiosarcomas are rare neoplasms. We here present the case of a 24 year old male with a cardiac mass which was characterised as malignant on 18F-Fluorodeoxyglucose (18F-FDG) positron emission tomography-computed tomography (PET-CT). In addition 18F-FDG PET-CT also demonstrated pericardial infiltration and bone metastases. The tumor was confirmed to be angiosarcoma on biopsy and palliative chemotherapy was started. Here we have highlighted the potential role of 18F-FDG PET-CT in patients with cardiac angiosarcoma and presented a brief review.

A recurrent activating PLCG1 mutation in cardiac angiosarcomas increases apoptosis resistance and invasiveness of endothelial cells.

Primary cardiac angiosarcomas are rare tumors with unfavorable prognosis. Pathogenic driver mutations are largely unknown. We therefore analyzed a collection of cases for genomic aberrations using SNP arrays and targeted next-generation sequencing (tNGS) of oncogenes and tumor-suppressor genes. Recurrent gains of chromosome 1q and a small region of chromosome 4 encompassing KDR and KIT were identified by SNP array analysis. Repeatedly mutated genes identified by tNGS were KDR with different nonsynonymous mutations, MLL2 with different nonsense mutations, and PLCG1 with a recurrent nonsynonymous mutation (R707Q) in the highly conserved autoinhibitory SH2 domain in three of 10 cases. PLCγ1 is usually activated by Y783 phosphorylation and activates protein kinase C and Ca(2+)-dependent second messengers, with effects on cellular proliferation, migration, and invasiveness. Ectopic expression of the PLCγ1-R707Q mutant in endothelial cells revealed reduced PLCγ1-Y783 phosphorylation with concomitant increased c-RAF/MEK/ERK1/2 phosphorylation, increased IP3 amounts, and increased Ca(2+)-dependent calcineurin activation compared with ectopic expressed PLCγ1-wild-type. Furthermore, cofilin, whose activation is associated with actin skeleton reorganization, showed decreased phosphorylation, and thus activation after expression of PLCγ1-R707Q compared with PLCγ1-wild-type. At the cellular level, expression of PLCγ1-R707Q in endothelial cells had no influence on proliferation rate, but increased apoptosis resistance and migration and invasiveness in in vitro assays. Together, these findings indicate that the PLCγ1-R707Q mutation causes constitutive activation of PLCγ1 and may represent an alternative way of activation of KDR/PLCγ1 signaling besides KDR activation in angiosarcomas, with implications for VEGF/KDR targeted therapies.

O1-10-2 - Patient Characteristics and Management of Primary Cardiac Angiosarcoma: National Cancer Center Hospital Experience

Background: Primary cardiac angiosarcomas are extremely rare and poorly understood. We reviewed the National Cancer Center Hospital (NCCH) experience of 7 patients with primary cardiac angiosarcoma to identify a preferred diagnostic and management strategy and evaluate the role of adjunctive therapy.Methods: We reviewed the patient database of NCCH to identify patients with primary cardiac angiosarcoma diagnosed in between 2008 and 2012. We identified 7 patients and reviewed the details of their symptoms, treatments and outcomes.Results: Of the 7 patients, 3 were male and 4 were female. The median age was 42 years old (range 34-60). The most common symptom of the patients at the first visit was dyspnea on exertion (n = 5) and peripheral coldness (n = 2), and all patients had pericardial effusion. In all cases, tumor originated in the right atrium. Four patients had localized disease and 3 patients had distant metastasis. Surgical resection was performed in 3 patients with localized disease. Complete resection was achieved in 2 patients, but all patients who received surgical resection had disease recurrence within 1 year. All patients with unresectable, metastatic or recurrent disease received systemic chemotherapy. They received weekly paclitaxel as first-line chemotherapy. Two of 7 patients (28.6%) achieved partial response (PR) and 4 of 7 patients presented stable disease (SD) with weekly paclitaxel. Median progression free survival was 4.8 months and median overall survival was 13 months.Conclusion: Primary cardiac angiosarcomas remain rare but lethal disease. The treatment response with paclitaxel for primary cardiac angiosarcoma in our institution is similar to the previously reported response in patients with angiosarcoma. Multidisciplinary therapy containing surgical resection and systemic chemotherapy may improve the clinical outcome of primary cardiac angiosarcoma.

Primary Cardiac Angiosarcoma with Adrenal Metastasis.

Cardiac angiosarcomas are rare tumours which can present with varying clinical symptoms, and are usually difficult to manage. We present a case of a young female who presented with respiratory symptoms, and was found to have a primary cardiac angiosarcoma with metastases. A discussion of primary angiosarcoma and a review of its management follows.

Cutaneous Metastases Can Be the Initial Presenting Sign of a Cardiac Sarcoma

To the Editors: We read with interest the authoritative CME article by Alcaraz et al1 on cutaneous metastases from internal malignancies published in the June issue. In their review, the authors describe the clinical, histopathologic, and immunohistochemical features of cutaneous metastasis from internal malignant neoplasms including breast, lung, gastrointestinal tract, oral cavity, genitourinary tract and thyroid carcinomas, carcinoid tumors, neuroblastoma, soft tissue sarcomas, and other origins embraced under the term “miscellaneous group.” A very uncommon origin of a cutaneous metastasis not mentioned by the authors is a cardiac sarcoma. Virtually all types of soft tissue sarcomas can be observed in the heart. However, these tumors are quite rare as only about 10% of surgically resected cardiac tumors are primary sarcomas.2 On the other hand, cardiac sarcomas presenting as metastatic disease are very rare.3 Angiosarcoma is the most common primary malignant neoplasm of the heart. In this, location depicts only 3% of angiosarcomas.4 This rare tumor has a predilection for middle-aged males and a marked predominance in the right side of the heart, specially the right atrium. The location in left atrium is rare because 78%–93% of cardiac angiosarcomas occur in the right atrium.5,6 The tumor commonly causes a nonspecific clinical presentation including congestive heart failure; precordial chest pain; dyspnea; constitutional symptoms of fever, weight loss, or malaise; cardiac murmurs, often of recent onset; and nonspecific electrocardiographic changes. Two-thirds of cardiac angiosarcomas are well- to moderately differentiated, showing vascular channels and papillary structures. However, the remaining one-third of these tumors are poorly differentiated or composed of spindle or anaplastic cells with poorly formed vascular channels.2 Metastatic spread at the time of diagnosis is frequent. The reported incidence of metastases varies from 66% to 89% of patients.5,6 Metastases occur most often to the lung, liver, lymph nodes, bone, adrenal gland, central nervous system, and spleen. Cutaneous metastases are very infrequent. A review of the literature yielded only 2 cases of primary cardiac angiosarcoma that presented as cutaneous metastases.7,8 Pomper et al7 described a 43-year-old man who initially presented with a soft tissue metastases below the left eye in the absence of cardiac symptoms. At surgery, the lesion was found to be invading the orbicularis muscle. The biopsy revealed a high-grade invasive angiosarcoma. One week postoperatively, a similar cutaneous lesion appeared at the superior border of the upper lip. Chest roentgenogram and computed tomographic scan revealed a right atrial mass. A biopsy of this mass showed a high-grade angiosarcoma consisting predominantly of spindle cells. We reported the case of a 51-year-old man who initially presented with multiple cutaneous metastases in the absence of cardiac symptoms.8 Physical examination revealed cutaneous nodules located in the right mandibular angle, left scapular region, thorax, and right hypochondrium. The nodules showed intact overlying skin; they were mobile and non-tender. The abdominopelvic computed tomographic scan revealed nodules in both lungs and liver. The skin biopsy was diagnosed as metastatic undifferentiated sarcoma. The tumor was relatively well circumscribed, was growing within the dermis and hypodermis, and was composed of cohesive islands with an epithelioid appearance. Tumor cells showed sharply demarcated eosinophilic cytoplasm and round to oval large nuclei containing one or two prominent nucleoli. There were moderate nuclear pleomorphism, mitotic activity, and necrotic areas. The stains for cytokeratin, desmin, muscle-specific actin, S100 protein, HMB45, CD45RB, and placental alkaline phosphatase were negative. The stain for CD31 showed intense positivity in the necrotic areas and in many tumor cells and was regarded as misleading. The patient died 26 days later with widely disseminated disease. At autopsy, a tumor arising in the wall of the left atrium and in the interatrial septum was found.8 After an immunohistochemical study including CD31 and CD34 stains, the diagnosis of cardiac pure epithelioid angiosarcoma was made. Unlike most cardiac sarcomas, our case manifested as an infiltrating tumor displaying auricular thickening not protruding or filling the chambers. It must be remembered that classification of cardiac sarcoma is difficult if there is limited tissue. Even in contemporary series, more than 20% of primary cardiac sarcomas are reported as unclassifiable.2,9,10 In conclusion, although cardiac sarcoma is a rare neoplasm, its presence should be suspected in patients with cutaneous metastatic sarcoma without an evident source of the tumor, even in absence of cardiac symptoms.

Primary Cardiac Angiosarcoma with Spontaneous Ruptures of the Right Atrium and Right Coronary Artery

Primary tumors of the heart are rarely seen. Cardiac angiosarcomas are malignant tumors that almost always have a poor prognosis. Atrium rupture and coronary artery fistula are very rare complications of primary cardiac angiosarcoma. We describe a 57-year-old man suffering from primary cardiac angiosarcoma with spontaneous ruptures of the right atrium and right coronary artery (RCA). Theoretically, either of these ruptures invariably results in pericardial effusion and tamponade that is rare but potentially life threatening. In this instance, however, the patient might have developed fibrous adhesions resulted from previous bloody pericardial effusion. A massive pericardial effusion was localized, which consequently prevented cardiac tamponade and hemodynamic collapse. Echocardiography revealed the tumor progression leading to detectable infiltration of solid mass into the right atrial (RA) wall, which is close to RCA. And color Doppler displayed the flow into the pericardial cavity through a disrupted RA wall and perforated RCA. Echocardiography remains the primary method of choice for evaluation of cardiac masses.

Cardiac angiosarcoma in a pregnant woman: a case report and review of the literature

Cardiac angiosarcomas, although very rare, are the most common primary malignant tumours. They are characterized by no specifi c clinical fi ndings, rapid growth with frequent metastasis at the time of diagnosis and a reduced response to treatment. We describe the case of a 34-year-old pregnant woman who began to complain of dyspnoea and palpitations at 32 weeks of gestation. The echocardiogram showed a mass in the right atrial cavity and the CT scan showed extensive local invasion and hepatic metastasis. After 4 weeks, because of progressive heart failure due to obstruction of the tricuspid valve by the mass, the patient was submitted to an urgent caesarean section followed, two days later, by palliative cardiac surgery in order to alleviate the symptoms. Adjuvant chemotherapy was performed.

Clinicopathologic and immunohistochemical characteristics of adult primary cardiac angiosarcomas: analysis of 10 cases

Primary cardiac angiosarcoma is a rare but the most common malignant neoplasm of the heart in adults. The objective of this study is to analyze the clinicopathologic characteristics of primary cardiac angiosarcoma. Ten cases of primary cardiac angiosarcoma treated in a single institution were analyzed for their clinical, pathologic, and immunohistochemical features. There were 6 men and 4 women, with a mean age of 40 years (range, 20-61 years). The patients commonly presented with dyspnea and distant metastasis. All tumors were located in the right atrium, with a mean tumor size of 6.8 cm. Tumors were hemorrhagic, with variegated tan-brown solid areas. Histologically, they exhibited high-grade morphology with mixed solid growth and anatomizing channels. Frequent mitoses and tumor necrosis were common. The tumors were strongly positive for CD31, CD34, FLI-1, and WT-1 but negative for AE1/3, D2-40, human herpesvirus 8, and epidermal growth factor receptor. The tumor cells were focally reactive to p53, with a high rate of Ki-67 expression. A complete tumor resection was not possible in any of the patients because of the size or extensive local invasion of the tumor. Overall survival ranged from 1 to 81 months (mean, 26.6 months) after initial histologic diagnosis. Primary cardiac angiosarcomas are rare tumors that commonly arise in the right atrium. The mean age is much younger than that of soft tissue angiosarcoma. Regional tumor extension and distant metastasis are extremely common at the time of diagnosis. Surgical resection with adjuvant chemotherapy is currently the preferred treatment, and survival time appears to be inversely correlated with the tumor size and degree of regional tumor extension at the time of surgery.

Angiosarcoma Arising from Right Atrium: Remarkable Response to Concurrent Chemoradiotherapy with Carboplatin and Paclitaxel

We read with interest the case report by Fehr et al.1Fehr M Kuhn M Mayer K et al.Metastatic angiosarcoma arising from the right atrium: unusual presentation and excellent response to treatment in a young patient.J Thorac Oncol. 2010; 5: 1301-1302Abstract Full Text Full Text PDF PubMed Scopus (3) Google Scholar and the concise review by Orlandi et al.2Orlandi A Ferlosio A Roselli M et al.Cardiac sarcomas: an update.J Thorac Oncol. 2010; 5: 1483-1489Abstract Full Text Full Text PDF PubMed Scopus (76) Google Scholar regarding cardiac sarcomas. Concurrent chemoradiotherapy (CRT) to cardiac angiosarcomas has been scarcely documented, and only one case was reported with the benefit from concurrent CRT with docetaxel.3Nakamura-Horigome M Koyama J Eizawa T et al.Successful treatment of primary cardiac angiosarcoma with docetaxel and radiotherapy.Angiology. 2008; 59: 368-371Crossref PubMed Scopus (28) Google Scholar Although efficacy of weekly paclitaxel (PTX) for unresectable angiosarcoma has been demonstrated,4Penel N Bui BN Bay JO et al.Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study.J Clin Oncol. 2008; 26: 5269-5274Crossref PubMed Scopus (387) Google Scholar CRT with weekly PTX was not reported before the report by Fehr et al. We present herein a case of metastatic angiosarcoma arising from the right atrium, which remarkably responded to concurrent CRT with weekly carboplatin and PTX. A 40-year-old man was referred to our institute, presenting a tumor in the right atrium and multiple bilateral lung nodules with the computed tomography (Figures 1A, B). Histology obtained by wedge resection of a lung nodule revealed a high-grade epithelial angiosarcoma, thus the patient was diagnosed with angiosarcoma from right atrium with multiple lung metastases. We judged it to be unresectable and decided to perform concurrent CRT (30 fractions of 2Gy) with weekly carboplatin (area under the curve = 2) and PTX (60 mg/m2) for 6 consecutive weeks. The radiation field was limited to the primary cardiac tumor. After the therapy, almost all bilateral pulmonary nodules disappeared, and a large tumor in the right atrium was markedly reduced (Figure 2A, B). Although grade 2 esophagitis and neutropenia were observed, the treatment was generally well tolerated. No evidence of progression has been confirmed after more than 5 months of follow-up.FIGURE 2A, Almost all bilateral pulmonary nodules disappeared 3 months after the initiation of chemoradiation therapy. B, The cardiac tumor in the right atrium was remarkably reduced 3 months after the initiation of chemoradiation therapy.View Large Image Figure ViewerDownload (PPT) Multimodality treatment combined with surgery and adjuvant chemotherapy and/or radiotherapy is indicated as the standard care for patients with cardiac angiosarcomas. Nevertheless, the majority of patients with cardiac sarcoma are already unresectable and metastatic disease at their initial diagnosis and have a poor prognosis. CRT is desirable in some cases with cardiac sarcoma to control both the primary cardiac tumor and metastatic lesions. In our case, a combined regimen including PTX was chosen to obtain better response of metastatic lesions, and good response was clearly observed. Moreover, the efficacy and tolerability of concurrent CRT with carboplatin + PTX have been recently demonstrated in locally advanced non-small cell lung cancer.5Yamamoto N Nakagawa K Nishimura Y et al.Phase III study comparing second- and third-generation regimens with concurrent thoracic radiotherapy in patients with unresectable stage III non-small-cell lung cancer: West Japan Thoracic Oncology Group WJTOG0105.J Clin Oncol. 2010; 28: 3739-3745Crossref PubMed Scopus (206) Google Scholar To our knowledge, this is the first report demonstrating the efficacy of concurrent CRT with carboplatin + PTX for cardiac angiosarcoma. Nevertheless, there is no other evidence of CRT with doublet regimens. Future studies to explore usefulness of concurrent CRT for cardiac angiosarcomas are warranted.

Right atrial angiosarcoma and electrocardiogram-gated cardiac computed tomography: a case report

Primary cardiac tumors are quite rare and most of these tumors are benign. In this report, a patient presented with chest distress and shortness of breath after activity. Echocardiography of other hospital showed a hyperechoic right atrial mass. Electrocardiogram-gated cardiac computed tomography (ECG-Gated CT) of our hospital provided accurate information about the site and extent of the tumor, and the involvement of neighboring structures, even about the malignant nature of the lesion. The pathological study indicated angiosarcoma. The role of ECG-Gated CT in the assessment of cardiac masses and tumors was discussed. Cardiac tumors are extremely rare and can be divided into benign and malignant lesions. Myxomas are the most common type of cardiac benign tumor, while angiosarcomas are the most common type of cardiac malignant tumor. Imaging studies play an important role in the diagnosis of cardiac angiosarcomas. Echocardiogram, computed tomography (CT) and magnetic resonance imaging (MRI) are the most common imaging studies for tumors evaluation. However, the precise tumor location is often difficult to evaluate precisely on the basis of two-dimensional source images. We conducted ECG-gated cardiac CT examination with 3D reconstruction for preoperative assessment in a patient with a angiosarcoma arising in the right atrium.

Primary Cardiac Angiosarcoma: A Case Report

Primary tumors of the heart are rarely seen. Cardiac angiosarcomas are malignant tumors that almost always have a poor prognosis. We describe a 29-year-old man with primary cardiac angiosarcoma with multiple site metastases. The therapeutic approach includes surgery, chemotherapy and radiotherapy alone or in combination. New techniques of radiotherapy and combined chemotherapeutic agents may relieve symptoms and prolong a patient's life. We discuss the diagnosis and treatment of cardiac angiosarcoma in the light of a case report.

Early detection and efficient therapy of cardiac angiosarcoma due to routine transesophageal echocardiography after cerebrovascular stroke

Primary malignant cardiac tumors (cardiac angiosarcomas) are exceedingly rare. Since there are initially nonspecific or missing symptoms, these tumors are usually diagnosed only in an advanced, often incurable stage, after the large tumor mass elicits hemodynamic obstructive symptoms. A 59-year-old female presented with symptoms of cerebral ischemia. A computed tomography (CT) scan showed changes suggestive of stroke. Transesophageal echocardiography revealed an inhomogeneous, medium-echogenic, floating mass at the roof of the left atrium near the mouth of the right upper pulmonary vein, indicative of a thrombus. At surgery, a solitary tumor was completely enucleated. Histologically, cardiac angiosarcoma was diagnosed. The patient received adjuvant chemotherapy and was free of symptoms and recurrence of disease at 14 months follow-up. Due to the fortuitous appearance of clinical signs indicative of stroke, cardiac angiosarcoma was diagnosed and effectively treated at an early, nonmetastatic, and therefore potentially curable stage. Although cardiac angiosarcoma is a rare disease, it should be taken into consideration as a potential cause of cerebral embolic disease.

Angiosarcoma Presenting as Syncope

A 31-year-old lady presented with anemia and syncope. Echocardiography revealed massive pericardial effusion with a right atrial mass. Transesophageal echocardiography, computed tomography and magnetic resonance imaging scans confirmed presence of a right atrial mass. Histopathology revealed a high grade angiosarcoma. Complete resection was done and the patient was referred to an oncology unit for further management. After three months the patient had extensive metastasis and succumbed to the disease. This case report highlights the clinical presentation, rapid and aggressive course of cardiac angiosarcomas.

Right Atrial Angiosarcoma: A Case Report

A 32-year-old female patient presented with dyspnea and palpitation, and transthoracic echocardiography revealed the presence of pericardial effusion. Pericardiosynthesis was performed for drainage. Because of the rapid accumulation of effusion and the presence of a right atrial mass on follow-up echocardiography, a computed tomography scan was done that revealed a right atrial defect and the presence of advanced pericardial effusion. The patient was prepared for an emergency operation. The mass on the right atrial wall was approached via a midsternal incision with cardiopulmonary bypass. The tumor filled the right atrial cavity, compressed vital structures, extended to the right ventricle, and had local metastases. As the tumor did not appear to be curable with surgery, a palliative approach was adopted. The right atrial free wall and tissues causing cardiac obstruction were totally removed, the tumor itself was partially excised, and local metastases were sampled. The resulting right atrial wall defect was closed with a Dacron patch. The operation ended uneventfully, and the clinical status and vital and hemodynamic findings of the patient returned to normal. The pathological diagnosis based on the samples obtained during the operation was angiosarcoma. The patient had an uneventful postoperative period and was then referred to an oncology center for clinical recovery. No findings of local recurrence or metastases were observed during the postoperative follow-up. The patient completed her combination therapy and currently is free of any clinical problems at her 13th postoperative month. We believe that advancements in radiotherapy and chemotherapy regimes combined with surgery (radical, if possible) for the treatment of cardiac angiosarcomas may provide better survival and quality-of-life results.

Intracardiac Mass in a Man Infected With Human Immunodeficiency Virus

Intracardiac Mass in a Man Infected With Human Immunodeficiency Virus