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Malignant Primary heart tumors are the tumors that originate from the heart tissue. Malignant form of cardiac tumors are extremely rare and account for 25% of the diagnosed cases. It is very hard to find information on cardiac sarcomas due to the limited data and the misdiagnosis with the other cardiac diseases. Most malignant heart tumors are sarcomas,which are very difficult to treat, and they have very poor prognostic results. The difficulty lies in the advanced tumor representation, the advanced stage at representation and due to the insufficient awareness of the disease . The course of the disease progression and its development is also not very well understood due to the same reasons mentioned earlier. There are many types of cardiac sarcomas. The most common type is Angiosarcoma, which has an incidence of 37% and is represented in male patients more than females. Other types of cardiac sarcomas include Pleomorphic undifferentiated sarcoma, Rhabdomyosarcoma, Leiomyosarcoma, Synovial sarcoma and intimal sarcoma, which is another type of soft tissue cancer in the blood vessels . The metastases to the heart from other tissues are up to 100 times more common than the primary heart tumors.

Mechanism and Causes

It is very hard to find a common reason for the development of heart sarcoma Some cytogenetic analysis revealed chromosomal changes and immunohistochemical analysis revealed high expression of p53 gene products, which contribute to the cell's ability to survive through any cellular damage leading to the uncontrolled division of the cell . However, some studies suggest reasons related to previous radiotherapy treatments. Current data are inconclusive regarding the cause of the cardiac tumors but suggest multiple factors that contribute to benign and malignant tumors of the heart. The associated factors include irregularities in the immune system, which compromises the body's ability to fight against any aberrant growths. Other factors include exposure to Radiation, Sun light, viruses and tobacco, which all contribute to the irregular growth of the cells causing tumor formation The current data suggest that complications of radiotherapy is associated with the development of cardiac tumors, Other data suggest recurrent inflammation that may contribute to most types of Angiosarcoma . The malignant tumor may grow at any site in the heart, and the location usually imposes the type of possible treatment. Some of the most common sites for tumor growth is the right atrium, which is the most common site for Angiosarcoma . Other locations include the Left Atrium and the bases of the large Aorta and the Pulmonary Artery . Spread and Metastasis of the disease is very common. The disease mostly spreads to the lungs. However, it is also possible for the disease to progress to the lymph nodes, bones, liver and the Central Nervous System (CNS) . The tumor formation may result in blockade of major blood vessels in the heart, based on its size or it may form an embolism that could travel through the blood to other organs and systems in the body and cause the manifestation of the symptoms .

Signs and Symptoms

The size and the locations of the tumor mostly dictates the types of the symptoms. The manifestations of the cardiac sarcomas mimic the signs and symptoms of the other cardiac diseases. These signs are often mistaken with coronary or vulvar cardiac disease. Most patients represent with symptoms of obstruction to blood flow and interference with valve function.

• Changes in body positions may trigger some of the symptoms of primary cardiac tumors and these may include Difficulty breathing when lying flat or when asleep, Chest pain or tightness in the chest, Palpitations or rapid heart rate, Fainting, lightheadedness or dizziness . Patients also suffer from irregular heart beats and arrhythmias as well as Pericardial effusion .
• Some patients suffer from emboli, including cerebral, coronary, and retinal emboli that represent detached pieces of the cancer traveling through blood to other tissues.
• Patients may also show up to the emergency room for the following symptoms:
  • shortness of breath,
  • Syncope
  • chest pain,
  • fever
  • malaise
• Other symptoms may mimic the symptoms of inflammation of the heart or Endocarditis ; These symptoms are general and may include
  • Fever or cough
  • Fingers that change color or turn blue Raynaud’s phenomenon , when pressure is applied.
  • Involuntary weight loss,
  • Joint pain, and swelling of the legs, ankles or abdomen can occur as well and are mistaken for Endocarditis but may indicate tumor of the heart in reality .

Diagnosis

The diagnosis of cardiac sarcomas happens usually at late stages of the disease. Much of the available data represent post-mortem analysis of the disease . However, it is believed that awareness and extra caution leads to better diagnosis of the disease. Cardiac sarcomas are best diagnosed via performing the necessary echocardiograph, which is proved to be the best tool for early diagnosis . Other tests include 3D echo, that is used for surgical procedure planning. Other diagnostic technologies that are used include Angiography, CT and MRI, which are found to differentiate between the benign and the malignant types of cancer .

Treatment and Prognosis

Due to the rarity and the late stage representation of the disease, the prognosis of cardiac sarcomas are considered to be dismal . Data show an overall mean survival rate of 12 months, few patients survived a mean of 27 months without recurrence. Some patients died of recurrent disease or Metastasis with no relevance for age on the onset of the disease . So far no valuable treatments have been proven to affect the course of the disease. However, the most common approach is a surgical approach . Tumor resection and valve replacement are found to be effective in improving the survival rate to few months. However, the tumor usually recur after resection. Autotransplatation of the heart and lungs is another approach. However, still this approach is not widely used due to the high chance of recurrence of the disease. The best current approach that is currently used but with limited resources is an adjuvant chemotherapy and radiotherapy after the surgical resection. The last approach is proven to improve the life expectancy of the patient to three years before the occurrence of the disease.

Recent Research

There is no record of ongoing research aimed towards prevention of the actual disease, or taking into consideration the course of disease progression, due to the rarity of the disease and to the limited available data. Most of the current research focuses on representation of case studies and aims on increasing the awareness about the primary cardiac tumors. The reason is because part of the major concern and the high mortality of the disease is based on misdiagnosis with other cardiac diseases, which are the results of unawareness. more studies are done to represent different scenarios on actual case studies with emphasis on the diagnostic approaches . A recent study suggested that misdiagnosis of the correct form of the disease is highly suggestive of the delayed treatment and the contribution to overall prognosis of the disease . Other studies focused mainly on the best approach towards treating the tumor. These studies emphasize on the most efficient ways for surgical and therapeutic interventions. A new research suggested that differentiating between the benign and the malignant forms of cardiac tumors before tumor resection is essential for the treatment and prognosis of the disease . The study also suggested the use of a combined diagnostic imaging technology of 18F-FDG PET/CT, which combines CT and PET scan as one diagnostic tool, suggesting the new technology as favorable diagnostic tool to differentiate between the different forms of the tumor. More studies and case reports are being published to emphasize on the importance of the disease. However, limited or no apparent results are shown to improve the prognosis of the actual disease .