Is There Heart Cancer? Types, Symptoms, Treatments

What is heart cancer? Is there heart cancer?

Yes, heart cancer does exist, but it’s an extremely rare form of cancer. Heart cancer, or cardiac sarcoma, is a primary malignant tumor that starts within heart tissues. 

But what is a heart tumor? Is it different from heart cancer?

A heart tumor, also known as a cardiac tumor, is a general term for all types of tumors in the heart. While most heart tumors are not cancerous, heart cancer is a primary cancerous heart tumor (1).

Heart tumors may begin within the heart or spread to the heart from other organs. They can be classified as primary and secondary heart tumors (2). Primary heart tumors develop in various parts of the heart and can be either benign (non-cancerous) or malignant (cancerous). They have an estimated prevalence of about 1 in 2000 autopsies (3).

Approximately 10 percent of primary cardiac tumors are malignant, and around 90 percent are benign (3). On the other hand, a secondary heart tumor can result from cancer spreading (metastasizing) to the heart from other organs. It is 20 times more common than primary tumors, with a prevalence of about 1 in 100 autopsies. 

Here’s how you can differentiate a heart cancer from a heart tumor.

Symptoms and types of heart cancer

Heart cancer is concerning due to its severity, so understanding its symptoms and types is crucial. Let’s explore what to look out for:

Heart cancer symptoms

The signs and symptoms of heart cancer vary depending on the location and size of the tumor (3, 4). Generally, its symptoms are often nonspecific and can be mistaken for other heart conditions.

Some people may experience mild symptoms or no symptoms at all, while others may have symptoms causing severe heart diseases. Here are some symptoms to watch for:

• Shortness of breath (dyspnea)
• Chest pain (angina)
• Swelling in legs and ankles (edema)
• Heart palpitations (irregular heartbeats)
• Fainting (syncope)
• Weight loss 
• Fatigue
• Pericardial effusion (fluid buildup causing pressure on the heart, also called cardiac tamponade)
• Fever
• Night sweats
• Raynaud’s phenomenon (fingers and toes turning numb and discolored)

Types of heart cancer

According to the 2021 WHO classification of heart tumors (5), the following are some types of heart cancer (malignant primary heart tumor):

• Sarcomas 
• Lymphomas

Cardiac sarcomas

Sarcoma is the rarest type of malignant heart tumor (around 20 percent of all primary tumors). It arises from the heart’s connective tissues, like muscle or fat (6). However, sarcomas are aggressive and can spread quickly. 

The following are the most common types of sarcoma:

• Angiosarcoma: This is the most frequently occurring malignant heart tumor, typically found in the right upper heart chamber (atrium) (7). It originates from the blood vessels and is known for its aggressive nature.
• Undifferentiated Pleomorphic Sarcoma (UPS): UPS, also known as malignant fibrous histiocytoma, is a rare form of cardiac sarcoma that develops in the left atrium (8). It is difficult to treat due to various cell shapes and sizes and lack of specific tissue origin.
• Leiomyosarcoma (LMS): LMS represents approximately 10 percent of cardiac sarcomas. It arises from the heart’s smooth muscle cells and is most often found in the left atrium (5).

Cardiac lymphomas

Lymphomas in the heart are less common and originate from the immune system cells within the heart (5). These include:

• Diffuse large B-cell lymphoma (DLBCL): The most common type of cardiac lymphoma, accounting for over 80 percent of cases. It usually affects older men.
• Fibrin-associated DLBCL (FA-DLBCL): A rare subtype of DLBCL, FA-DLBCL grows within blood clots in the heart and is often incidentally discovered during medical procedures. It can be associated with Epstein-Barr virus infection (an infection spread through saliva).

Types of benign primary heart tumor

According to the WHO, benign primary heart tumors are also classified as follows (5):

Myxoma 

Cardiac myxomas are benign tumors that usually form in the left atrium of the heart in adults. They can vary in size and shape, sometimes appearing bumpy or finger-like (9).

Myxomas can cause problems like strokes, heart attacks, fatigue, or weight loss. Myxomas in other heart chambers or those in multiple locations might indicate a genetic condition called Carney complex.

Fibroma 

Fibroma is the second most common heart tumor found in children. It primarily arises in the ventricular septum (the wall separating the heart’s chambers) (10). These tumors consist of fibroblasts and collagen fibers and can sometimes cause irregular heartbeats.

Rhabdomyoma 

Rhabdomyoma is the most common cardiac tumor in children, with many cases linked to tuberous sclerosis (11). Rhabdomyomas are hamartomas, which are disorganized growths of normal tissue, in this case, cardiac muscle cells. These large cells have empty spaces inside, forming distinct, rounded lumps resembling spiderwebs under a microscope.

Adult cellular rhabdomyoma 

Unlike the more common pediatric rhabdomyoma, adult cellular rhabdomyoma occurs in adults and doesn’t have “spider cells.” It’s a rare tumor that looks similar to tumors outside the heart and is usually well-defined and cellular (12).

Lipoma 

Cardiac lipomas are encapsulated (surrounded by a protective outer layer) masses of mature fat cells that can occur in any heart layer. It manifests as a fatty lump in the heart (13). 

Another similar tumor, lipomatous hypertrophy of the atrial septum, occurs when the wall between the two upper heart chambers thickens with fat tissue. The difference between these tumors is that the former is encapsulated, while the latter is an unencapsulated mass of cells.

Lipomatous hamartoma of the atrioventricular valve 

While not cancerous, these fat cells grow in the heart’s valves (5). They can thicken the valves, giving them a bumpy, nodular appearance. 

The valves are crucial for controlling blood flow through the heart, and any changes in their structure could potentially affect heart function.

Hamartoma of mature cardiac myocytes 

These are lumps of disorganized heart muscle cells, sometimes containing fat, nerves, or blood vessels, in the lower heart chambers (ventricles) (14). They lead to inefficient blood pumping from the heart to the body and lungs.

Mesenchymal cardiac hamartoma 

Like the previous type, a mesenchymal cardiac hamartoma consists of different cell types, including muscle, fat, and nerves (5). Because of this mix, the growth is called ‘mesenchymal,’ which refers to the connective tissue from which these cells originate. These hamartomas can disrupt the normal rhythm of the heart and may lead to serious heart rhythm problems or sudden cardiac death in some cases.

Conduction system hamartoma (histiocytoid cardiomyopathy)

Previously known as “histiocytoid cardiomyopathy,” conduction system hamartoma is now recognized as being more tumor-like than a cardiomyopathy (5). 

The heart has a special wiring system that controls its beat. In the conduction system hamartoma, this wiring becomes tangled due to small, yellow-brown lumps that grow along it. 

These lumps are made of unusual, large cells and may disrupt the function of energy production cells. This can cause an enlarged heart, irregular heartbeats (arrhythmias), and even sudden death, especially in young people.

Hemangioma

Hemangioma appears as a non-cancerous lump in your heart made of tangled blood vessels (15). There are different types, but they all involve thin-walled vessels that can sometimes cause problems:

• Capillary hemangioma is a network of tiny blood vessels.
• Cavernous hemangioma is a collection of larger, spongy blood vessels.
• Arteriovenous hemangioma is a direct connection between arteries and veins, bypassing capillaries.

These lumps usually grow in the heart muscle walls and might not cause any issues. At times, they can lead to shortness of breath, fainting, or chest pain.

Cystic tumor of atrioventricular node

This rare heart condition is present at birth (congenital) and involves a tiny cyst near the heart’s control center (atrioventricular node) (16). Even though the cyst is small, it can cause big problems, including sudden death. The cyst lining is made up of different cell types, but these cells themselves aren’t abnormal.

Secondary heart tumor

Secondary heart tumors are more common than primary ones (17). Common cancers that metastasize to the heart include:

• Lung cancer
• Melanoma (skin cancer)
• Breast cancer
• Kidney cancer
• Leukemia
• Lymphoma (different from primary cardiac lymphoma, as it starts elsewhere in the body)

Heart cancer survival rate

How long can you live with a heart tumor or cancer? The survival rate for heart cancer and heart tumors can be based on several factors, including: 

• Type of tumor (benign or malignant)
• Size and location of the tumor
• Stage of the tumor (if malignant)
• Your age and overall health
• Availability and effectiveness of treatment options

Primary heart cancers are generally aggressive and have a poor prognosis, while benign heart tumors may have a better outcome if they can be surgically removed. For primary malignant heart tumors, the one-year survival rate is 46 percent, dropping to 22 percent at three years and 17 percent at five years (18). 

A recent study published in December 2023 found that the overall survival rates of primary cardiac metastasis at various time points post-diagnosis were as follows (19):

• 30-day survival rate: 85 percent
• 3-month survival rate: 59 percent
• 6-month survival rate: 51 percent
• 12-month survival rate: 38 percent

Malignant tumors have a poor prognosis compared to benign. However, with improvements in treatment options, the survival rate has increased over the years. Benign tumors like myxomas usually have a good outlook after surgery, with most people returning to normal lifespans. 

However, it’s important to note that everyone is different, and these statistics may not reflect every person’s experience. For the most accurate and personalized information, consult your doctor.

Can you get heart cancer or a heart tumor? Who will be at risk?

While anyone can develop heart cancer, certain risk factors can contribute to the development of heart cancer or heart tumors:

• Age: People aged 50 and over have a higher chance of developing heart cancer (20).
• Gender: Males have a slightly higher risk of heart cancer than females (38.2 versus 30.0 cases per 100 million persons) (20).
• Race: White individuals are more likely to get heart cancer compared to other races (21). 
• Genetic susceptibility: Some people may have a genetic susceptibility to heart tumors. Specific conditions associated with increased risk include:
  • Family history of cardiac tumors
  • Carney complex (22) (benign myxomas)
  • Tuberous sclerosis (23) (benign cardiac rhabdomyoma)
  • Gorlin syndrome (24) (benign cardiac fibroma)
  • Mutations in certain genes (e.g., MDM2, PDGFRA, CDKN2A) (25) (cardiac sarcoma)
• Secondary heart tumors: People that have cancer in other regions of the body, such as the lungs, kidneys, skin, stomach, breast, and blood.

Heart tumor symptoms

Heart tumors can be benign or malignant, as described above. They can arise from any heart tissue and affect heart function through their presence and growth (26). Watch out for these symptoms of a heart tumor:

• Obstruction of blood flow: Similar to heart cancer, tumors can block blood flow in the heart chambers, leading to shortness of breath, fatigue, and chest pain. Furthermore, tumors growing on or near heart valves can cause valve malfunction, mimicking valve diseases.
  
• Changes in heart sounds: A tumor might cause abnormal sounds due to disrupted blood flow, which can be detected by a stethoscope.
  
• Arrhythmias: Tumors can interfere with the heart’s electrical pathways, causing irregular heartbeats.
  
• Constitutional symptoms: Fever, malaise, and weight loss can occur, especially with malignant tumors.
  
• Embolism: Pieces of the tumor can detach and travel through the bloodstream, which may cause strokes.
  
• Pericardial effusion: Fluid accumulation around the heart can compress it, resulting in cardiac tamponade, a life-threatening condition.

What are heart failure symptoms? 

Heart failure is a chronic condition in which the heart struggles to pump blood efficiently throughout the body. This can lead to a variety of signs that may progressively worsen as the heart’s function declines. Being aware of these indicators is essential for prompt medical intervention.

According to the American Heart Association (AHA), the following are the symptoms of heart failure (27):

• Difficulty in breathing 
• Chronic coughing or wheezing produces mucus that may be white or tinged with pink, indicating blood
• Swelling in the ankles, legs, or abdomen
• Fatigue
• Appetite loss or nausea
• Cognitive impairment (confusion or memory problems) 
• Rapid heartbeat
• Weight fluctuations

These symptoms occur because the heart cannot pump enough oxygen-rich blood to meet the body’s needs. If you notice any of these symptoms, consult a doctor for a thorough evaluation and an appropriate treatment plan.

Exams and tests

Doctors use a variety of tests to accurately diagnose heart cancer or cardiac tumors (28, 29). The following are common diagnostic tools:

Clinical evaluation

• Medical history and physical exam: The first step in diagnosing a heart tumor is a thorough medical history and physical exam. Your doctor will ask about your family history, symptoms, and any risk factors you might have. A physical exam will follow, where your doctor checks for signs that might suggest heart problems.

Imaging studies

• Echocardiography: This is an initial test used to visualize the heart’s structure and function. It can detect masses within the heart chambers or on the valves.
• Cardiac MRI: It provides detailed images of the heart structure and can differentiate between benign and malignant cardiac masses.
• CT scan: It provides cross-sectional images of the heart and can help determine the size and location of the tumor.
• PET scan: It is used to detect cancerous cells in the body, including the heart, and is often used alongside a CT scan.

Laboratory tests

• Blood tests: Certain blood tests, including tests for tumor markers that might be elevated in the presence of cancer, can help diagnose heart tumors.
• Biopsy (endomyocardial biopsy): If a tumor is suspected based on imaging studies, a biopsy may be performed to obtain a tissue sample to examine under a microscope.

Other tests

• Electrocardiogram (ECG): This test records the electrical activity of your heart and shows any irregularities due to the tumor. ECG can detect about 90 percent of cardiac tumors (30).
• Cardiac catheterization: A catheter is inserted into your heart to obtain detailed information about its function and to take tissue samples if needed.

Genetic testing

• Molecular genetic testing: This involves analyzing your DNA to identify any inherited genetic mutations that might increase your risk of developing heart cancer.

Treatments

Heart cancer, or primary malignant cardiac tumor, is a serious condition that requires comprehensive treatment. 

Currently, there is no specific treatment for heart cancer due to a lack of clinical trials. However, doctors may consider the following options depending on your condition and tumor type (31).

Surgery

Surgery is the primary treatment for most cardiac tumors, especially benign ones. The goal is to remove the tumor completely, which can cure benign tumors like myxomas.

For malignant tumors, surgery aims to remove as much of the tumor as possible. However, complete removal may not always be feasible due to the tumor’s location or size.

Chemotherapy and radiation therapy

Chemotherapy and radiation therapy are often the primary treatments for cancer. These treatments help to shrink the tumor size, manage symptoms, and increase survival rates. 

Radiation therapy is often used in combination with chemotherapy and other anti-cancer drugs.

Targeted therapy

Targeted therapy involves drugs that specifically target cancer cells without affecting normal cells. This treatment is used for certain heart cancers with specific genetic markers.

Palliative care

Palliative care is an important aspect of treatment, focusing on improving the quality of life for patients with serious diseases. It includes managing symptoms and providing support to patients and their families.

Monitoring and follow-up

Regular monitoring and follow-up are crucial to check for the recurrence of the tumor and manage any long-term effects of the treatment.