مقالات پذیرفته شده در هفتمین کنگره بین المللی زیست پزشکی
Cardiac Angiosarcoma: A Review of Clinical Symptoms, Diagnosis, Treatment, and Prognostic Factors
Cardiac Angiosarcoma: A Review of Clinical Symptoms, Diagnosis, Treatment, and Prognostic Factors
Mahdi Zarei,1,*
1. Research Center for Evidence-Based Medicine, Tabriz University of Medical Sciences, Tabriz, Iran
Introduction: Cardiac tumors are a group of rare neoplasms that are associated with a high mortality rate. Most of these neoplasms are primary cardiac tumors (PCTs). Among them, patients with malignant primary cardiac tumors (MPCTs) usually have lower survival and higher mortality rates. Most malignant primary cardiac tumors are sarcomas, with one-, three-, and five-year survival rates of 46%, 22%, and 17%, respectively, reported in these patients. Angiosarcoma is one of the most common cardiac sarcomas, accounting for about half of all sarcomas. This malignancy is slightly more common in men and is usually diagnosed in patients aged 39 to 48.9 years with non-specific clinical symptoms. Despite the invasive nature of this malignancy and the high mortality rate in these patients, there is no specific guideline for timely diagnosis or appropriate treatment. Therefore, the aim of this study is to review the clinical symptoms, appropriate diagnostic modalities, effective treatment approaches, and determining the factors affecting the prognosis of these patients.
Methods: For this review study, a comprehensive search was conducted in PubMed, Web of Science, Scopus, and Google Scholar databases. After removing duplicate and non-English articles, cross-sectional, case-control, and case series articles were included in the study and review articles were excluded. Initially, to examine the thematic relationship of the articles, their titles and abstracts were evaluated, and after removing irrelevant articles, their full text was examined. The information from the articles was extracted and combined and compared with each other to obtain results.
Results: Cardiac angiosarcoma accounts for more than 50% of all cardiac sarcomas. The incidence of this malignancy is slightly higher in men and the average age of diagnosis is 44.4 years. It mainly presents with non-specific symptoms such as dyspnea, pericardial effusion, and chest pain, which is why most patients have been diagnosed with metastases to the lungs, liver, and bones at the time of diagnosis. The most common anatomical location of tumor is the right atrium, but it can also invade the pericardium, lymph nodes, and superior vena cava. The mortality rate of this malignancy is 64.7 to 100%, and most of these tumors are grade 3 or higher on pathological diagnosis. According to studies, the most effective diagnostic modalities for the tumor are MRI, Transthoracic Echocardiography, or CT scan, and the most suitable modalities for diagnosing metastasis are CT scan, MRI, or PET/CT scan. The most effective treatment approach that has also been associated with increased patient survival, depending on the patient's condition, is Radical Resection with adjuvant chemotherapy. In some studies, radiotherapy has also been considered as a suitable approach to improve patient survival. The most important factors affecting the prognosis of the disease are age over 45 years, tumor size larger than 5 cm, local invasion and metastasis at the time of diagnosis, tumor histological grade, and the presence of tumor necrosis and cytogenetic aberrations such as 1q+.
Conclusion: Cardiac angiosarcoma is a rare malignancy that is associated with low survival rates and high mortality. This disease is accompanied by nonspecific clinical symptoms that can lead to delayed diagnosis and metastasis. Despite the invasive nature of this tumor, there is no suitable guideline for diagnosis, treatment, and prognosis of this disease. Knowledge of clinical symptoms, available diagnostic modalities, and appropriate treatment approaches can help physicians manage it. Finally, the development of multicenter clinical studies to identify effective diagnostic and therapeutic modalities, as well as laboratory studies to better understand the oncologic markers of this malignancy, seems essential.