Pericardial Mesothelioma

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It's one of the rarest types of asbestos-related cancers. Treatment options are few because the heart lining, which is where tumors develop, is too close to the delicate organ; however, there are some cases where surgery can help remove small tumors.

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Pericardial mesothelioma develops in the thin membrane surrounding the heart, known as the pericardium. The membrane has two layers: An outer layer called the parietal layer, heart sac or theca cordis; and an inner layer known as the visceral layer or epicardium.

There are four common forms of mesothelioma, and pericardial and testicular are the rarest. To date, approximately 200 cases of pericardial mesothelioma are described in medical literature — that's 1 percent of all known diagnosed mesotheliomas.

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Almost all mesotheliomas can be traced to asbestos exposure, but medical researchers continue to study the link between asbestos and cancer of the pericardium. Studies are clear on how inhaled microscopic fibers reach the lungs, but less clear on how the fibers reach the heart.

This form of cancer strikes twice as many men as women, and is most often diagnosed in people between the ages of 50 and 70. Like the other types of mesothelioma, the disease develops over a long period of time — one to five decades — and is typically discovered at a later stage. Symptoms include chest pain, fatigue and shortness of breath. Diagnosis is difficult because symptoms mimic those of other disorders.

Pericardial treatment options: Surgery (if a patient is deemed healthy enough), chemotherapy, radiation therapy or a combination of all three modalities. About half of the people who contract the pericardial type survive longer than six months.

What Are the Symptoms of Pericardial Mesothelioma?

What Are the Symptoms of Pericardial Mesothelioma?

Most pericardial mesothelioma patients experience no symptoms when the cancer initially develops, a fact that contributes to a late-stage diagnosis. The symptoms also resemble those of other heart conditions, making the cancer difficult to accurately diagnose. Most symptoms are caused by fluid buildup around the heart and thickening of the pericardial layers.

Most symptoms are caused by the buildup of fluid and the thickening of pericardial layers. The presence of any of these symptoms should be followed by a visit to the doctor with recommended screenings such as an X-ray or CT scan.

The following symptoms may indicate pericardial mesothelioma:

  • Heart palpitations or irregular heartbeat (arrhythmia)

  • Difficulty breathing, even when at rest (dyspnea)

  • Murmurs
  • Cough
  • Shortness of breath when lying flat

  • Chest pain
  • Fatigue
  • Fever
  • Night sweats

If you are experiencing any of these symptoms, especially heart-related symptoms, schedule an appointment with your doctor immediately. Screening tests like X-rays, CT scans and echocardiograms can usually reveal the underlying cause of these health problems.

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How Does Asbestos Cause Pericardial Mesothelioma?

The causal relationship between asbestos exposure and this type of mesothelioma is not fully understood. Researchers confirm the pleural and peritoneal types of the cancers are primarily caused by exposure to asbestos, yet the causes of the pericardial type are less definitive. Some patients with this rare type of mesothelioma have a history of exposure, but the limited number of reported cases prohibits researchers from effectively analyzing the relationship between the two. A 1982 study found a history of asbestos exposure in three of 15 cases, and a 1994 study reported asbestos exposure in four of 15 cases.

Pericardial mesothelioma is thought to occur when asbestos lodges itself around the heart.

Asbestos fibers can become lodged in the membranes that surround the heart. When this occurs, just as it does in the linings of the lungs and abdomen, the body experiences extreme difficulty in eliminating the fibers. They can remain stuck in the membranes, and over a long period of time — sometimes up to 50 years — the asbestos fibers cause the cells to undergo changes that may result in cancer.

Once cells become cancerous, they begin to divide more rapidly, without the restraint that regulates the growth cycles of healthy cells. As the mesothelioma cells continue to grow, they cause the lining of the heart to thicken, and eventually tumors develop. These changes in the membrane lead to the buildup of fluid between the pericardial layers, which combined with the thickening of the layers, puts pressure on the heart.

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How Is Pericardial Mesothelioma Diagnosed?

To diagnose pericardial mesothelioma, doctors make a cumulative assessment of your symptoms, medical history and current medical condition. Next, you will receive a physical examination, imaging tests and biopsy to determine the location of tumors and confirm whether or not they are cancerous.

When you see a doctor to discuss heart-related symptoms, one of the first diagnostic tests you will receive is an echocardiogram, which is essentially an ultrasound for your heart. This noninvasive test uses sound waves to help doctors see the size and shape of your heart and determine how well it's working overall.

Although an echocardiogram can reveal fluid buildup around the heart and help guide pericardiocentesis, the procedure doctors perform to drain the fluid, other imaging scans are needed to determine if potential tumors are present. If doctors spot abnormal growths, they need to take a fluid or tissue sample and perform a biopsy, which can confirm a mesothelioma diagnosis.

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Physical Examination

A small percentage of all heart-related mesothelioma diagnoses, between 10 and 20 percent, are made before a patient dies. Some of the challenges with early and accurate diagnosing of the pericardial type include the minuscule number of people with the disease and the delayed presentation of symptoms.

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Imaging Tests

Radionuclide imaging is an advanced detection method involving the injection of radiogallium, a radioactive material, to identify cancerous cells. It was reportedly used to detect at least one case of this type of cancer. Patterns of radioactivity show possible tumor sites in the human body.

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A biopsy is a crucial diagnostic procedure that can be more suitable in determining the cancer's point of origin. A surgeon will remove tissue or fluid from the pericardium, examine its cells under a microscope to positively confirm the presence of pericardial mesothelioma.

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Computed tomography (CT) scans are effective for detecting pericardial tumors, making them a preferred diagnostic tool. Magnetic resonance imaging (MRI) can also yield extremely successful results in identifying and assessing the cancer. These tumors generally are not localized, and they tend to cover most of the heart. Furthermore, this cancer type accounts for approximately half of all pericardial tumors.

Seek a Second Opinion to Avoid Misdiagnosis

Because pericardial mesothelioma symptoms like chest pain, cough and difficulty breathing are shared with various other diseases, it's crucial to meet with a specialist to confirm your diagnosis. To ensure an accurate diagnosis and prompt treatment, you need to speak with an expert who knows the intricacies of this rare cancer.

Pericardial mesothelioma is often mistaken for more common heart illnesses, including:

  • Heart failure
  • Coronary heart disease
  • Tuberculosis pericarditis
  • Constrictive pericarditis
  • Cardiac tamponade
  • Intra-atrial myxoma
  • Cardiomyopathy

If you have been diagnosed with any of these conditions, always seek a specialist to confirm. An accurate and early diagnosis not only gives you access to a wider range of treatment options, but it may also lead to improved survival.

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What Treatments Are Available?

Treatment options are limited because the heart lining rests so closely to the heart itself that most therapies can easily damage this delicate organ. Most patients with pericardial mesothelioma are not ideal candidates for surgery, which is often the most effective treatment for this cancer. However, there are rare cases where the cancer is diagnosed early and surgery can be carried out to remove small, localized tumors. Still, any surgery near this area is extremely dangerous because of the close proximity to the heart.

The four primary treatment options for pericardial mesothelioma are pericardiectomy, chemotherapy, palliative treatment and fine needle aspiration.

  • Pericardiectomy

    Patients who doctors decide are good candidates for surgery are treated with a pericardiectomy, the surgical process of removing part or all of the pericardium. A pericardiectomy can relieve pressure and minimize fluid buildup, allowing the heart to continue functioning properly. A pericardiocentesis removes excess fluid from the pericardium to relieve pressure around the heart.

  • Chemotherapy

    Chemotherapy is an option that is normally considered for asbestos-related cancers, but the clinical benefits seen in pericardial patients who received this treatment were minimal. Similarly, radiation therapy, which is considered one of the primary therapies for most peritoneal and pleural cases, is considered minimally effective for this rare cancer.

  • Palliative Treatment

    Palliative treatment options are the most viable and common treatments, which aim to minimize pain and reduce the symptoms caused by the buildup of fluid in the pericardium. Essentially, palliative care can improve quality of life and make patients more comfortable. Palliative treatments can include therapies that relieve pain and pressure around the heart, such as a pericardiocentesis or pain medication.

  • Fine Needle Aspiration

    Another treatment that removes excess fluid is known as a fine needle aspiration. This palliative procedure is more commonly used as a diagnostic method for pleural mesothelioma patients, but can provide comfort for certain patients.

What Is the Average Prognosis?

The common prognosis for the pericardial type is poor, compared to that of peritoneal or pleural mesothelioma. Nearly 50 to 60 percent of pericardial patients pass away within six months of receiving a diagnosis. However, this is not the case for everyone.

Hope exists for some patients because researchers have cited positive results after surgery to remove local tumors. In one study, surgery to remove part of the heart's lining combined with radiation therapy improved survival in two patients.

One patient lived a year after treatment, and another was alive five years after treatment. Smaller, less impactful benefits were demonstrated from chemotherapy.

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Early diagnosis can yield a more optimistic prognosis, often resulting in more treatment options.

Additional Resources

Karen Selby is a registered nurse and a Patient Advocate at The Mesothelioma Center. She worked in several subspecialties within nursing before joining in 2009.

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