Clinical and Imaging Features of Primary Cardiac Angiosarcoma

This study aims to explore computed tomography (CT) and magnetic resonance imaging (MRI) features of patients diagnosed with primary cardiac angiosarcoma. The study involved the analysis of 12 patients diagnosed with primary cardiac angiosarcoma who underwent non-contrast (8/12) or contrast-enhanced CT (10/12) or MRI (4/12). Imaging appearances, including the tumor location and adjacent infiltration, were analyzed. All 12 lesions were located in the right atrium with a broad base. Adjacent invasion including the tricuspid valve and right ventricle (2/12), inferior or superior vena cava (2/12), pericardium (10/12), and right coronary artery (7/12) was common. On unenhanced CT scans, tumors in two patients were homogeneous in density, whereas the others were inhomogeneous. Ten patients showed heterogeneous enhancement. The enhancement pattern showed no direct correlation with the di erentiation degree of the tumor. Four lesions manifested as heterogeneous intensity, with hyperintense hemorrhage foci on both T1- and T2-weighted MRI. Three showed rim enhancement and one showed patchy enhancement. Primary cardiac angiosarcoma often involves the right side of the heart with infiltration of peripheral structures. CT features include typical inhomogeneous density on unenhanced scans and heterogeneous centripetal enhancement on enhanced scans. A cauliflower-like appearance on both T1- and T2-weighted MRI is common. The characteristic enhancement pattern of MRI remains to be determine

Diagnosis and treatment of cardiac angiosarcoma

Primary cardiac tumors are very rare in the population. About 20% of it are malignant. The most common type of cardiac malignant tumor is angiosarcoma. This tumor has the ability to infiltrate surrounding tissues and form distant metastases. Angiosarcoma is most often located in the right atrium and it mostly attacks men. This neoplasm is difficult to diagnose, because it has no specific symptoms. Patients with this tumor may complain of a chest pain, cardiac arrhythmias, suffocation and distal oedema. The rarity of occurrence and the lack of specyfic symptoms contribute to late diagnosis of the disease and worse prognosis of patients. Angiosarcoma can also be misdiagnosed as pneumonia or more common cardiovascular disease for example coronary artery disease. In advanced stage, this tumor can even lead to myocardial rupture. Various imaging methods are used in the diagnostics of this neoplasm for example: CT, MRI, PET-CT or Echocardiography. Histopathological examinations are less frequently used due to the risk of serious complications like hemothorax. Due to the very uncommon occurrence of the angiosarcoma, guidelines describing treatment of patients with this neoplasm, have not been developed yet. Currently, surgical removal of the tumor (if it is possible), chemiotherapy and radiotherapy are used. Reaserches on the genetic mutations which cause angiosarcoma and the development of appropriate drugs may give patients a chance for more effective treatment in the futur

Primary Cardiac Angiosarcoma: A Fatal Disease

A 42-year-old man with a cardiac tamponade underwent an urgent pericardiotomy that showed tumoral tissue, covering the surface of the right atrium. The tumor was then partially excised, and the histological examination revealed the presence of a moderately-differentiated angiosarcoma. The patient was then referred to the oncology unit and scheduled for a chemotherapy schedule including Epirubicin (60 mg/m2, on days 1 and 2) plus Ifosfamide (2000 mg/m2, on days 1 to 3) and Uromitexan (2000 mg/m2 at hours 0, 4, 8 after IFO). All drugs were administered every three weeks. After two cycles, a restaging work-up revealed a partial remission. The treatment was continued for another two cycles. A new evaluation by cardiac MRI evidenced a local and distant (lung) progression of disease. The patient died after three months. This paper confirms that cardiac angiosarcoma is a fatal disease, and the prognosis is usually 6–11 months from time of diagnosis

Angiosarcoma Cardíaco. Uma Revisão

Based on a case of a patient with angiosarcoma (AS) of the right atrium with superior vena cava syndrome associated with urticaria and polyarthralgias, who died soon after surgery, the authors present a brief review of the subject of cardiac AS, an extremely rare pathology, usually diagnosed late due to its non-specific symptomatology. Several topics are discussed, including mechanisms of clinical manifestations caused by blood flow obstruction and valve dysfunction, local invasion with arrhythmias and pericardial effusion, embolic phenomena and constitutional symptoms. Imaging and histopathologic methods of diagnosis are considered, as well as references to cytogenetic analysis. Surgery is the first treatment choice, but heart AS are frequently not completely resectable and concomitant metastases at the time of surgery are common, both usually leading to a dismal prognosis. Chemotherapy, radiotherapy and even heart transplantation do not substantially improve the survival of these patients. Urticaria is not generally assumed by most authors to be associated with malignancy, but there are rare reports of its association with some malignant tumors

Cardiac tumors in a tertiary care cancer hospital: clinical features, echocardiographic findings, treatment and outcomes

Cardiac tumors are a rare entity, comprised of tumors with diverse histology and natural history. We report the clinical characteristics, echocardiograhic findings, therapy and outcome of 59 patients with primary and metastatic cardiac tumors. Our institutional echocardiogram data base from 1993 through 2005 was reviewed to identify patients diagnosed with intra-cardiac tumor. A total of 59 patients with cardiac tumors were identified and included in the study. The patients' characteristics, presenting symptoms, diagnostic tests, location, histology of the tumor, treatment and the one year survival rate of this population was collected from the medical records. Of the 59 cardiac tumor cases, 16 (27%) were primary cardiac tumors and 43 (73%) were secondary cardiac tumors. The most common primary tumor was sarcoma affecting 13 (81%) of the 16 cases. Of these, 5 patients were angiosarcoma, 5 unclassified sarcoma, one myxoid sarcoma and 2 malignant fibrous histiocytoma. The mean age at presentation was 41.1 years, and the most common location was right atrium affecting 6 cases (37.5%). The most common symptom of dyspnea was present in 10 (62.5%) cases. Eleven (25.6%) of the 43 secondary cardiac tumors were metastasis from renal cell carcinoma. The mean age at presentation was 55.4 years. Right atrium was the most frequent location affecting 18 (42%) of the 43 patients. The most common presenting symptom was dyspnea in 15 (35%) cases. For both primary and secondary tumors, dyspnea was the most common symptom and right atrium was most frequently involved. Sarcoma was the most common primary cardiac tumor while metastasis from renal cell carcinoma was the most common secondary tumor

Primary Cardiac Angiosarcoma

Primary cardiac sarcoma is a rare clinical entity, with an incidence of 0.0001% in collected autopsy series. The majority of the literature describes a uniformly dismal prognosis with a median survival of only 6 months for these aggressive tumors. Standard surgery, adjuvant chemotherapy, and radiotherapy have been consistently unsuccessful. Early heart transplantation and novel radiation therapy approaches may offer a survival benefit in nonmetastatic tumors, but up to 80% of the patients present with systemic metastasis at diagnosis. Though several chemotherapeutic regimens have been tried, the role of chemotherapy is not well established and outcome data available is minimal. Liposomal doxorubicin (PLD) has been shown to be useful in the treatment of soft tissue sarcomas, and our case supports its use in cardiac angiosarcoma

Imaging features of rare mesenychmal liver tumours: beyond haemangiomas.

Tumours arising from mesenchymal tissue components such as vascular, fibrous and adipose tissue can manifest in the liver. Although histopathology is often necessary for definitive diagnosis, many of these lesions exhibit characteristic imaging features. The radiologist plays an important role in suggesting the diagnosis, which can direct appropriate immunohistochemical staining at histology. The aim of this review is to present clinical and imaging findings of a spectrum of mesenchymal liver tumours such as haemangioma, epithelioid haemangioendothelioma, lipoma, PEComa, angiosarcoma, inflammatory myofibroblastic tumour, solitary fibrous tumour, leiomyoma, leiomyosarcoma, Kaposi sarcoma, mesenchymal hamartoma, undifferentiated embryonal sarcoma, rhabdomyosarcoma and hepatic metastases. Knowledge of the characteristic features of these tumours will aid in guiding the radiologic diagnosis and appropriate patient management

Cardiac Masses on Cardiac CT: A Review

Cardiac masses are rare entities that can be broadly categorized as either neoplastic or non-neoplastic. Neoplastic masses include benign and malignant tumors. In the heart, metastatic tumors are more common than primary malignant tumors. Whether incidentally found or diagnosed as a result of patients’ symptoms, cardiac masses can be identified and further characterized by a range of cardiovascular imaging options. While echocardiography remains the first-line imaging modality, cardiac computed tomography (cardiac CT) has become an increasingly utilized modality for the assessment of cardiac masses, especially when other imaging modalities are non-diagnostic or contraindicated. With high isotropic spatial and temporal resolution, fast acquisition times, and multiplanar image reconstruction capabilities, cardiac CT offers an alternative to cardiovascular magnetic resonance imaging in many patients. Additionally, cardiac masses may be incidentally discovered during cardiac CT for other reasons, requiring imagers to understand the unique features of a diverse range of cardiac masses. Herein, we define the characteristic imaging features of commonly encountered and selected cardiac masses and define the role of cardiac CT among noninvasive imaging options

Intracardiac tumors: Results from a single heart center

Background: This study aims to present the results of surgical treatment of intracardiac tumors in our cardiac surgery center. Methods: Data of 21 patients with intracardiac tumors (15 males, 6 females; median age 60.9 years; range 35 to 87 years) who underwent surgery between April 2006 and May 2015 were retrospectively analyzed. The patients were diagnosed preoperatively by transthoracic echocardiography and computed tomography or magnetic resonance imaging. Results: The mean follow-up was 27.85 months. Of 21 patients, 12 had benign tumors and nine had malignant tumors. Of the benign tumors, nine were myxomas, one was an intramuscular lipoma, one was an interatrial septal lipoma, and one was a papillary fibroelastoma. Of the malignant tumors, eight were metastatic tumors and one was a primary tumor. Median sternotomy was performed in all patients to access the heart. Thrombi due to a cardiac mass were detected intraoperatively in some patients. Conclusion: Our study results suggest a high-degree of diagnostic confusion between intracardiac thrombi and tumors. Therefore, metastatic cardiac tumors should be considered in patients with pleural or pericardial effusion of no other identified cause