What are the other Names for this Condition? (Also known as/Synonyms)

• Haemangioendothelial Sarcoma of Heart
• Malignant Haemangioma of Heart
• Primary Angiosarcoma of Heart

What is Primary Cardiac Angiosarcoma? (Definition/Background Information)

• Primary Cardiac Angiosarcoma is an infrequent and aggressive, but often rapidly developing tumor of the heart that presents no signs and symptoms in the early stages. It is known as a primary tumor, since it first arises in the heart; later on, it can involve or spread to other parts of the body including the lungs and liver
• Angiosarcomas are regarded as a type of high-grade malignant cancer that spreads and infiltrates the surrounding tissues and organs. “High-grade” means that the tumor is very aggressive
• Children and adults of a wide age category are known to be affected by Primary Cardiac Angiosarcoma. The specific risk factors and cause of Cardiac Angiosarcomas are not well-established
• The signs and symptoms due to the tumor that is normally found in the right atrium, may include chest pain, anemia, and bleeding episodes (including easy bruising)
• In many cases, the signs and symptoms of Primary Cardiac Angiosarcoma may be non-specific, due to which the tumor is detected at an advanced stage (large-sized tumor), when metastasis may have already occurred
• Combinations of surgery, radiation therapy, and/or chemotherapy are used in the treatment of Primary Cardiac Angiosarcoma. During later stages, it is difficult to entirely remove the tumor, as it is locally aggressive and can infiltrate into adjoining heart tissues
• The prognosis for an individual with Primary Cardiac Angiosarcoma is very poor, due to its high metastasizing potential. A majority of the individuals die within 9-10 months following diagnosis of the tumor

Who gets Primary Cardiac Angiosarcoma? (Age and Sex Distribution)

• Primary Cardiac Angiosarcoma is the most common malignant tumor of the heart; even though malignant tumors of the heart by itself are a rare occurrence
• It affects a wide range of age groups (between 3-80 years), but it normally peaks around the age of 30-40 years
• Some studies say that males are affected more than females, though some indicate an equal prevalence
• There is no known ethnic or racial preference

What are the Risk Factors for Primary Cardiac Angiosarcoma? (Predisposing Factors)

The risk of Primary Cardiac Angiosarcoma may be linked to the following factors:

• A previously performed radiotherapy for other cancer conditions - the tumor may be radiation-induced. In such cases, it is called radiation-induced angiosarcoma. This is also referred to as “secondary”, because it is caused by the radiation that treated the original tumor. The radiation has an expected side effect of damaging the genetic material in the normal cells. This can lead to the development of a “secondary” cancer
• Chronic obstruction of the lymph vessels, called lymphedema, due to mastectomy (surgical removal of the breast or the lymph nodes under the arm pit; a treatment for breast cancer). This can increase the risk of developing soft tissue angiosarcoma. Similarly any condition that causes lymphatic blockage of the heart, may increase the risk for developing Primary Angiosarcoma of Heart
• Chemical and environmental contaminants, such as arsenic, thorium dioxide, vinyl chloride,  dioxin, and many other agents
• Presence of foreign materials in the body, such as graft material (plastic, steel), bone wax, polyester fiber (Dacron, from sutures), etc., which are sometimes known to act as carcinogenic (cancer-causing) agents
• Angiosarcoma of soft tissue has been associated with genetic conditions including neurofibromatosis (type 1), Klippel-Trenaunay syndrome, and Maffucci syndrome. Such syndromes have the potential to increase the risk for Primary Cardiac Angiosarcoma, even though a definitive association has not yet been determined
• Individuals affected by immune deficiency disorders, such as AIDS, have a higher risk of developing the tumor

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Primary Cardiac Angiosarcoma? (Etiology)

• The exact cause and mechanism of formation of Primary Cardiac Angiosarcoma is unknown
• They are thought to occur as a reactive process owing to some preexisting factor(s), which may include:
  • Radiation exposure
  • Chronic lymphedema
  • Exposure to chemicals and environmental pollutants
  • Presence of foreign materials in the body for long periods (such as an implant or graft)

What are the Signs and Symptoms of Primary Cardiac Angiosarcoma?

The signs and symptoms of Primary Cardiac Angiosarcoma depend upon factors such as the following:

• Tumor size
• Tumor location in the heart
• Extent of involvement and spread

The signs and symptoms of Primary Angiosarcoma of Heart may include:

• In nearly 50% of the cases, chest pain is the most common indication. However, labored breathing is not noted initially
• Right-sided heart failure may occur, since most tumors form on the right atrium
• Arrhythmias are usually present
• Pericardial effusion
• The tumor may lead to bleeding events including easy bruising and impaired clotting ability of blood
• Other features include anemia, the presence of hematoma, and cardiac tamponade
• Invasion into adjoining valves or compression of valves may occur
• In over 1 in 10 individuals, non-specific symptoms, such as fever, weight loss, and fatigue, not attributable to any cause/reason may be observed; this is another factor for a delayed diagnosis of the malignant tumor

The following features of Primary Cardiac Angiosarcoma may be observed:

• Most tumors are present in right atrium (60-80% cases), on the atrial wall; they may be found in other heart chambers too, but rarely in the left atrium
• The involvement of pericardium is often noted
• The mass can be irregular and nodular, with size ranging from 2 cm to larger sizes
• Cardiac Angiosarcomas are mostly poorly-differentiated, but may be well-to-moderately-differentiated too
• The malignant tumors initially grow at a moderate rate, and then suddenly start progressing rapidly, during the later stages. In most cases, the initial tumor growth periods are asymptomatic
• Due to large size of tumor growth, the adjoining organs, nerves, and muscles, may be compressed or restricted. Often, these signs may be the first indications of the tumor

How is Primary Cardiac Angiosarcoma Diagnosed?

A diagnosis of Primary Angiosarcoma of Heart may involve the following:

• Complete evaluation of family (medical) history, along with a thorough physical examination; including examination of the heart (with special emphasis to signs such as abnormal heart sounds)
• CT scan or MRI scan of the heart with IV contrast: A right atrial nodular mass with features of invasion and the presence of pericardial effusion is considered to be indicative of angiosarcoma
• Electrocardiogram (EKG): It is used to measure the electrical activity of the heart, in case of arrhythmias
• Transthoracic echocardiography (TTE): This procedure uses sound waves to create a motion picture of the heart movement
  • It can help show the size and shape of the heart and how well the chambers and valves are working
  • It can also help visualize the tumors, especially the large tumors; 1 mm and lesser-sized tumors may not be visible though
• Doppler ultrasound: Sound waves are used to measure the speed and direction of blood flow
• Tissue biopsy of the tumor:
  • A tissue biopsy of the nodule is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis
• PET scans may be used to determine the extent of tumor spread in the body

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Primary Cardiac Angiosarcoma?

The complications of Primary Cardiac Angiosarcoma may include:

• Congestive heart failure, depending on the location of the tumor in the heart
• Increased risk for thromboembolism (blood clot obstructing a blood vessel)
• Metastasis:
  • Usually by the time the angiosarcoma is detected, it is likely that it has grown and metastasized aggressively
  • Spread of tumor to the lungs may be noted in 70% of the cases, followed by metastasis to the liver
  • 90% are known to have metastasized at the time of diagnosis
• High recurrence rate, even after surgical excision and removal; inaccessibility of the tumor makes it hard to be completely removed
• Blood loss during invasive treatment methods may be heavy
• Damage of vital nerves, blood vessels, and surrounding structures during surgery
• Side effects from chemotherapy (toxicity), radiation therapy

How is Primary Cardiac Angiosarcoma Treated?

The treatment of Primary Cardiac Angiosarcoma may include:

• Any combination of chemotherapy, radiation therapy, and invasive procedures (surgery), may be used treat Angiosarcomas of Heart
• Surgery: Complete excision where possible is attempted. However, it is hard to remove the tumor fully, and so, a partial removal is generally made, which can help obtain a temporary relief from the signs and symptoms
• Embolization (clotting the vessels in the tumor) may be used to provide temporary relief from the symptoms and reduce blood loss during a surgical procedure
• Heart transplantation may be undertaken in some rare cases; nevertheless, it does not significantly alter the prognosis
• Follow-up care with regular screening and check-ups are important

How can Primary Cardiac Angiosarcoma be Prevented?

There is no definitive method to prevent Primary Cardiac Angiosarcoma occurrence. However, general precautions that decrease the incidence of cancer in the body may be considered, which include:

• Adopting a lifestyle that avoids prolonged exposure to pollutants and carcinogens, either at home or at work
• Due to its high metastasizing potential and high recurrence rate, regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already been treated for this tumor

What is the Prognosis of Primary Cardiac Angiosarcoma? (Outcomes/Resolutions)

• Primary Cardiac Angiosarcomas are rare, but highly malignant tumors. The prognosis depends on a combination of factors, such as:
  • Age of the individual
  • Tumor size and location
  • Its Ki-67 value - a protein found in cells that is a good indicator of how fast the tumor cells are growing. The Ki-67 value is determined by a pathologist and is usually mentioned in the pathology report
  • Response to treatment and medical therapy
• In general, the prognosis of Primary Cardiac Angiosarcoma is very poor. 90% of those affected are known to die within the first 9 months (or earlier)
• Some reports indicate that following removal of the tumor and the use of other treatment therapies, the survival period may be about 10 months
• The factors that adversely affect the prognosis of Cardiac Angiosarcoma include:
  • Late diagnosis of the condition; usually when the tumor is at an advanced stage (and metastasis has occurred)
  • Poor response to treatment
  • Complete removal of the tumor is very difficult and so recurrence after surgery is always noted

Additional and Relevant Useful Information for Primary Cardiac Angiosarcoma:

• Primary tumors of heart are rare and they account for only 5% of heart tumors. Metastatic tumors to the heart are far more common
• Angiosarcomas are classified into three major types:
  • Angiosarcoma of Skin
  • Angiosarcoma of Bone
  • Angiosarcoma of Soft Tissue