Pericardial mesothelioma is extremely rare. It is caused by asbestos fibers lodged in the lining of the heart. This results in tumors in the pericardium, the protective lining around the heart. Some patients have extended their life expectancy more than a year with the right treatment.
Understanding Pericardial Mesothelioma
There are three main types of mesothelioma: peritoneal, pleural, and pericardial mesothelioma. Primary malignant pericardial mesothelioma is the rarest type.
Only 200 cases of pericardial mesothelioma have been reported in medical journals. This represents about 1% of all mesothelioma diagnoses — a very rare incidence. Few veterans are ever diagnosed with pericardial mesothelioma.
There are two layers of the pericardium, an inner and an outer layer. Mesothelioma can develop in either layer. Experts consider asbestos to be the cause of mesothelioma. Yet, they can't explain how asbestos fibers get into the pericardium.
Doctors only know that asbestos fibers have the ability to migrate once inside the body. This also makes it hard to determine the cause of pericardial mesothelioma in veterans. Most veterans are exposed to asbestos in the military, but it’s not certain this could have been the cause of their diagnosis.
Pericardial mesothelioma is twice as common in men compared to women. Some experts think this is because men are more likely to get exposed to asbestos in the workplace.
Most people with this disease are 50-70 years of age when diagnosed. It often takes several decades before the asbestos exposure develops into mesothelioma cancer. Pericardial mesothelioma is a difficult disease to diagnose because of its rarity. It's also difficult to diagnose because the symptoms are similar to those found in more common diseases.
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Symptoms of Pericardial Mesothelioma
There are few to no symptoms when the disease remains localized to the pericardium. This is why it is rarely diagnosed in its early stages.
When symptoms do occur, they look like those seen in heart disease. As a result, people are often diagnosed with some type of heart condition before doctors find the mesothelioma.
The telltale symptoms of pericardial mesothelioma are usually found with visual imaging tests. One common sign is pericardial fluid buildup around the heart. This is often detected with an echocardiogram, X-rays or a CT scan of the chest.
Typical symptoms seen in pericardial mesothelioma include:
- Chest pain
- Dry cough
- Difficulty breathing (dyspnea)
- Exhaustion
- Fever
- Heart palpitations
- New onset of a heart murmur
- Night sweats
- Shortness of breath (with or without activity)
- Weight loss
- Worsening of shortness of breath when lying down
If you have any of these symptoms, it is important to seek medical attention. These signs could show that you have fluid around your heart. If you know you have been exposed to asbestos, your symptoms could be a result of pericardial mesothelioma.
Causes of Pericardial Mesothelioma
Doctors and researchers have not yet found the exact cause of pericardial mesothelioma. Based on the other forms of mesothelioma, asbestos exposure is the most likely the biggest risk factor and only known cause of the disease.
The exact relationship between asbestos and pericardial mesothelioma is less clear than it is in more common kinds of mesothelioma.
Theoretically, asbestos fibers could travel from the lungs to the lining around the heart. The asbestos fibers embed into the lining around the heart, irritating the tissue. It can take several decades for the asbestos fibers to migrate to the pericardium and turn the tissues into cancerous cells.
After pericardial cells mutate and turn into a malignancy (cancer), they divide quickly and form tumors around the heart. The cancer cells thicken the pericardial lining and fluid begins to build up in response to the irritation of the lining. This puts added stress on the heart, leading to heart-related symptoms.
Diagnosis of Pericardial Mesothelioma
During diagnosis, a doctor will first note your symptoms and perform a physical exam.
Imaging tests, such as an echocardiogram, a magnetic resonance imaging (MRI) scan, a chest X-ray or a computed tomography (CT) scan, may show thickening of the pericardium.
Tests like MRIs and CT scans can also reveal fluid around the heart.
Specialized doctors called thoracic surgeons may then perform a procedure called a pericardiocentesis. Your doctor inserts a needle into the space between the heart and the pericardium. This allows the doctor to take a fluid sample. Sometimes cancer cells can be found when looking at this fluid under the microscope.
In some cases, it takes a biopsy of the affected area to definitively make the diagnosis of pericardial mesothelioma.
Another technique uses radioactive dye to find pericardial mesothelioma. An injection of this dye can show evidence of the disease on imaging tests. However, CT scans of the chest are still the most common imaging test. CT scans show the fluid around the heart and possibly the cancer itself.
Only about 10-20% of pericardial cases are diagnosed while the patient is still alive. The rest are found in an autopsy after a mesothelioma patient has already died of the disease.
Pericardial mesothelioma is often misdiagnosed as:
- Acute heart failure
- Cardiac tamponade
- Coronary artery disease
- Intra-atrial myxoma
- Pericarditis
- Tuberculosis of the pericardium
- Weakness of the heart muscle
If you were diagnosed with any of the above conditions and you suspect a misdiagnosis, you should seek a second opinion from a heart and lung specialist.
They will be able to make sure the diagnosis isn’t something less common, such as pericardial mesothelioma.
Treatment of Pericardial Mesothelioma
Mesothelioma of the pericardium is difficult to treat because of the cancer's proximity to the heart.
There are several methods used to treat the disease:
- Chemotherapy is often the first line of treatment. Doctors also use chemotherapy to kill tumor cells that weren’t removed by radiation and surgery. Cisplatin is a commonly used chemotherapy treatment for this cancer.
- Radiation therapy can shrink the size of pericardial tumors and relieve symptoms.
- Surgery can remove the visible signs of a tumor. The most common surgery for this mesothelioma is called a pericardiectomy. This involves surgical resection of the heart lining.
- Palliative treatments can also be used to ease symptoms. For example, minor treatments can remove fluid buildup around the heart (pericardial effusion) and reduce pericardial thickening.
- New treatments continue to be studied in clinical trials. One treatment called immunotherapy has been approved to treat pleural mesothelioma — it may also become available to pericardial mesothelioma patients at some point.
Surgery is the best chance for prolonging a poor prognosis. A 2010 study showed that one patient was still alive 16 months after their operation. This treatment effectively quadrupled the patient’s life expectancy.
Sadly, pericardial mesothelioma is so rare that doctors have little evidence of effective treatments to compare.
Researchers wrote about this in a 2017 analysis of pericardial mesothelioma treatments. “Primary pericardial mesothelioma is a rare cancer, for which there is no consensus on treatment,” they said.
The lack of consensus makes it worth considering clinical trials and emerging therapies. Some patients live years past their diagnosis after getting drugs from a clinical trial.
About 50% of people diagnosed with pericardial mesothelioma survive past the six-month mark. This means the cancer has a very low median survival.
Yet, this also means half of the patients out there may be benefiting from their treatment. Only a mesothelioma specialist can determine the best course of treatment that can improve survival time.
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Taking Control of Your Diagnosis
Doctors have identified several factors that affect a mesothelioma prognosis, including age, sex, and how advanced the disease is. Another factor is the treatment you receive.
Treatment is probably the most important factor in life expectancy. More importantly, it’s a factor that you can take control of.
Important points for veterans with pericardial mesothelioma:
- A prognosis can change over time
- Consider being a part of clinical trials
- Seeing a specialist is a must
Remember that there isn’t a standard of treatment for pericardial mesothelioma. Take this into account when considering your treatment options. Every patient is different, and there are always people living longer than before. Learn more in our free veterans packet.
