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Papillary Fibroelastoma - Causes, Symptoms, Diagnosis, and Treatment

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Papillary fibroelastoma is a benign tumor that occurs in the heart. Read below to know in detail about this condition.

Medically reviewed by

Dr. Muhammad Zohaib Siddiq

Published At March 21, 2023
Reviewed AtJuly 11, 2023

What Is Papillary Fibroelastoma?

A papillary fibroelastoma is a benign or noncancerous type of heart or cardiac tumor and is the most common type of primary heart tumor. Though papillary fibroelastoma is a noncancerous condition, it can be dangerous and causes complications by traveling to other parts of the body. In most cases, the papillary fibroelastomas occur in the heart valve. People above 40 years are mostly affected, and it rarely affects children. It affects 1 in 2000 people each year. In 95 percent of the cases, the tumor is located in the left side of the heart and, in rare cases, grows from the lining of the heart chambers.

The aortic valve of the heart is mostly affected. The aortic valve is among the four valves in the heart that connects the left ventricle (the chamber that pumps blood from the heart to the body) and the aorta (the artery that carries blood away from the heart). The aortic valve is present on the left side of the heart. The functions of the valve are not affected due to papillary fibroelastoma.

What Causes Papillary Fibroelastoma?

The main cause of this condition is not known, but it is believed that it is caused if any damage occurs to the endothelial cells. The endothelial cells make the lining of the blood vessels, and these cells are in direct contact with the blood. When a signal is passed from the endothelial cells, the growth and development of the connective tissues forming the layer of the blood vessels are organized. When the endothelial cells are damaged, the blood clots form together and move to the site where the damage occurred. In some cases, the clots may occur in different areas of the body that can cause severe complications.

What Are the Symptoms of Papillary Fibroelastoma?

In most cases, there are hardly any symptoms. Still, some of the symptoms that can be a sign of this condition include:

  • Fainting or weakness. The patient may frequently faint even after taking good rest.

  • Shortness of breath. An individual may feel suffocating even just by walking or going from one place to another.

  • Transient ischemic attack (also known as mini-stroke is like a stroke but lasts for minutes to sometimes hours).

  • Angina (a type of chest pain).

How Is the Body Affected Due To Papillary Fibroelastoma?

The blood clots that travel through the bloodstream are triggered and travel to various parts of the body. However, it mainly affects the organs like kidneys, lungs, brain, spinal cord, coronary arteries, and retina of the eye. If the blood clots reach these organs, it can lead to heart attack, ventricular fibrillation (malfunction of normal heart pumping), or stroke. In severe cases, if the blood does not reach the heart through the coronary artery, it can lead to cardiac arrest.

What Are the Modalities Through Which Papillary Fibroelastoma Is Diagnosed?

The symptoms are rarely noticeable in this condition, so papillary fibroelastoma is mostly diagnosed while testing for some other condition or if a doctor is examining the patient to check for the cause of a stroke.

  • Imaging Tests: Magnetic resonance imaging (MRI) is an imaging test done to diagnose any certain tumor, and in such cases, papillary fibroelastoma is diagnosed as an underlying condition. It also uses magnetic waves and a computer to record the images. During the examination, the machine generates a squeaky and sharp noise that may cause irritability, so earplugs are given to block the noise. The examination takes half an hour to one hour to complete. Along with an MRI, another imaging test, computed tomography (CT) scan, is done to check for the exact size of the tumor. It also uses high magnetic waves and is rotated on the different angles of the affected area to get a more clear picture of the tumor.

  • Echocardiogram: Once the papillary fibroelastoma is diagnosed in an imaging test, an echocardiogram is done to understand the condition better. It is an ultrasound test that checks the function of the heart. There are many ways through which an echocardiogram is done, and two-dimensional (2D) ultrasound is most commonly used. Through high magnetic waves, the images appear as slices on the computer screen.

A transesophageal echocardiogram is done to diagnose the small tumors in the body. It also uses sound waves to capture the images. A long thin tube known as an endoscope guides the transducer down the throat, and the food tube or esophagus makes the sound waves, and the picture is then shown on the computer screen. The tumor will appear as a very small ball as it is less than one centimeter in size.

What Are the Treatment Options for Papillary Fibroelastoma?

If the tumor is diagnosed, the best treatment option is to surgically remove the tumor surgically to reduce the risk of tumor recurrence. An incision is given in the chest, the tumor is removed carefully, and the heart is again checked carefully to see if any tumor is still present.

If the surgical method is risky in some cases, anticoagulants or blood thinners are given to remove the blood clots. The blood thinners are given for a particular time period that helps in removing the clots to form together.

Both these treatment options need to be closely monitored as there are chances of the tumor recurrence.

Conclusion

Papillary fibroelastoma is a noncancerous tumor that affects the heart and is usually seen in people above 40 years of age. There are no visible symptoms in most cases, but if any mentioned similar cases are seen, then they should be immediately checked as the tumor can spread to different parts of the body. It is diagnosed as an underlying condition while testing for some other disorder. Once it is diagnosed, it can be treated surgically or through blood thinner. Patients should be monitored closely after the treatment to check if the tumor has recurred again or caused any complications.

Frequently Asked Questions

1.

Is a Non-cancerous Tumor Significant?

Generally, non-cancerous tumors are not serious. The seriousness can be due to the enlarging size and tumor location. Some non-cancerous tumors cause symptoms, including obstruction and pain. Sometimes, the non-cancerous tumor might be converted into a cancerous one, spread to other parts, or put pressure on the surrounding structures.

2.

Do Non-cancerous Tumors Need to Be Pulled Out?

Generally, non-cancerous tumors do not cause any harm to an individual. The tumor removal is not required until it causes pain, discomfort, or pressure. But sometimes, if the non-cancerous tumor can cause complications, such as in the case of a brain tumor, it may enlarge in size and compress the skull, causing brain issues. Sometimes, the tumor needs to be pulled out due to aesthetic concerns. The other reason for removal is the chance of converting into malignant tumors.

3.

Can Non-cancerous Tumors Be Treated?

Non-cancerous tumors can be treated with medical and surgical interventions. The treatment methods include:
- Complete Surgical Removal: The main line of non-cancerous tumor treatment includes full surgical removal of the tumor by preserving the structures that are present around the tumor.
- Medical Treatment: The non-cancerous tumor can sometimes be treated with medications. The medications will shrink the tumor and provide relief from symptoms. 

4.

What Is the Life Expectancy of a Non-cancerous Brain Tumor?

The life expectancy of a non-cancerous brain tumor is different in all individuals. Typically, the non-cancerous do not cause any harm, do not move from their original place, and do not spread to other places. Mostly, non-cancerous brain tumors are treated with surgery and medical treatment. Sometimes, the tumor may reoccur after surgery. 

5.

Can an Individual Live a Normal Life With a Brain Tumor?

The impact of living with a brain tumor is based on the tumor category, whether the tumor is benign or malignant. The other considerations related to the patient's survival with the tumor are as follows:
- The symptoms include pain, discomfort, seizures, and headaches. If an individual consults a healthcare provider on time for treatment interventions, these interventions will help in living a normal life.
- The tumor's presence may affect an individual speech, memory, and concentration.
- The treatment side effect may affect normal function.
- Rehabilitation and support programs will help in living a normal life.

6.

What Happens to an Individual if the Benign Tumor Is Not Removed?

Generally, the benign tumor does not require any treatment unless it causes any harm to an individual. However, there are some situations where a non-cancerous or benign tumor can cause problems.
- Some benign tumors increase in size and put pressure on the structures that are present around them.
- The tumors can cause obstruction and inhibit normal function.
- Hormonal imbalances.
- Aesthetic concern.
- Sometimes, the chance of converting into malignant tumors.

7.

Can a Non-cancerous Tumor Convert to a Cancerous Tumor?

Yes, a non-cancerous tumor can convert into a cancerous one. The benign tumors that can change into cancerous are moles into melanoma and benign breast tumors to breast cancer. So, regular follow-up and monitoring of non-cancerous tumors are essential to detect the high risk of malignant transformation.
 
 

8.

Why Does a Tumor Keep Increasing in Size?

The growth of the tumor is based on tumor category, whether it is non-cancerous or cancerous. 
 
The factors that lead to the continuous growth of the tumor are as follows:
- Tumor cells proliferate, multiply, and divide very rapidly. Due to the multiplication of the tumor cells, there is uncontrolled cell growth. This phase is called cell proliferation.
- Tumors lead to the generation of new blood vessels, which will help nourish the tumor with oxygen and nutrient supply.
- Tumor cells will help build the body's immune system, causing the tumor cell to survive. 
- Genetic mutations in the tumor cells.
- Tumor cells invade normal tissues and organs.

9.

Are Non-cancerous Tumors Cause Pain on Touch?

Generally, non-cancerous tumors do not cause pain on touch. But some people feel pain in touch. The factors that can lead to pain in a benign tumor are as follows:
- Location on sensitive areas.
- The large size of tumors.
- Local inflammation due to the tumor.
- Nerve compression.
- Vascular tumors cause pain.
- Infected benign tumors.

10.

Do Non-cancerous Tumors Run in Families?

The non-cancerous tumors run in families. 
 
The conditions include:
- Neurofibromatosis (nerve non-cancerous tumor).
- Multiple endocrine neoplasia (endocrine gland cancer).
- Tuberous sclerosis complex (benign tumor contributing body organs).
- Von Hippel Lindau disease (non-cancerous tumor in the brain).

11.

What Is a Non-cancerous Tumor That Starts in the Heart on the Aortic Valve?

The non-cancerous tumor involves heart valves, particularly the aortic valve. The size of this tumor is very small and appears in a sea anemone shape. The non-cancerous tumor does not cause symptoms but can cause some complications, including valvular dysfunction and coronary artery obstruction.

12.

What Side of Aortic Valve Is Affected With Fibroelastoma?

The rare non-cancerous tumor that is present on the aortic valve side. The tumor can be present on any cusp of the aorta valve. The size can cause complications in the aorta valve movement and cause complications. Surgical removal is the recommended treatment to restore normal and healthy function.
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Dr. Muhammad Zohaib Siddiq
Dr. Muhammad Zohaib Siddiq

Cardiology

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