Pericardial Mesothelioma

Expert Fact Checked

This page was medically reviewed by James Stevenson, M.D. on February 22, 2019. For information on our content creation and review process read our editorial guidelines. If you notice an error or have comments or questions on our content please contact us.

James Stevenson, M.D. Thoracic Medical Oncologist

Pericardial mesothelioma is a rare form of cancer. The cancer develops in the lining of the heart and accounts for about 1% of mesothelioma cases.  It has a poor prognosis, and on average, patients survive about six months after diagnosis. However, research is ongoing to find effective treatments for this rare mesothelioma.

01. What Is Pericardial Mesothelioma?

What Is Pericardial Mesothelioma?

Pericardial mesothelioma is an extremely rare type of cancer that forms in the lining of the heart, called the pericardium.

Cardiac (heart) tumors are very rare. When they do occur, it is often a result of metastasis from cancer elsewhere in the body. However, pericardial mesothelioma has not been widely linked to the metastasis of cancer in other parts of the body.

In the case of pericardial mesothelioma, there is anecdotal evidence of other conditions (such as pleural empyema) masking the cancer.

Fewer than 200 cases of pericardial mesothelioma have been reported. With so few cases, research is limited. Researchers’ understanding of life expectancy and the most effective treatment continues to evolve.

02. Pericardial Mesothelioma Causes

What Causes Pericardial Mesothelioma?

Due to the lack of reported pericardial mesothelioma cases, research around what causes this disease is limited. However, researchers have determined potential risk factors for pericardial mesothelioma.

Potential Pericardial Mesothelioma Risk Factors

  • Simian virus 40 infection
  • Radiation exposure
  • Thorotrast (a dangerous contrast agent historically used in certain medical scans)
  • Tuberculosis
  • Erionite exposure

Asbestos Exposure and Pericardial Mesothelioma

Pericardial mesothelioma has been linked to asbestos exposure. Other forms of mesothelioma, such as peritoneal and pleural mesothelioma, are caused by asbestos fibers.

Asbestos exposure may occur while at work, home, school or many other locations. Asbestos was widely used before the 1980s. As a result, it can be difficult to pinpoint where and when exposure took place in any mesothelioma case.

Inability to detect asbestos exposure can make identifying the cause of pericardial mesothelioma cases very difficult.

03. Pericardial Mesothelioma Symptoms

Symptoms of Pericardial Mesothelioma

Pericardial mesothelioma symptoms are often related to the heart muscle and can be vague. Symptoms may also take many years to present. In some cases, the symptoms are never diagnosed. In these instances, pericardial mesothelioma may not be diagnosed until autopsy.

Pericardial mesothelioma develops in the layers of membrane around the heart. Due to the involvement of the heart, patients may experience symptoms at early stages.

Possible Pericardial Mesothelioma Symptoms
  • Dry cough
  • Pericardial effusion
  • Pericardial thickening
  • Chest pain
  • Fatigue
  • Cardiac tamponade
  • Cardiomyopathy
  • Right shoulder pain
  • Shortness of breath (dyspnea)
  • Dysphagia (difficulty swallowing)
  • Swelling of the legs or lower extremities

Pericardial mesothelioma symptoms are vague and easily attributed to other conditions. This can lead to a difficult diagnostic process and the potential for misdiagnosis.

04. Diagnosing Pericardial Mesothelioma

How Is Pericardial Mesothelioma Diagnosed?

Malignant pericardial mesothelioma can be diagnosed with a combination of methods, including:

  • Echocardiogram
  • PET scan
  • CT scan
  • MRI
  • CT-guided liquid biopsy
  • Cytology
  • Histology
  • Immunohistochemistry
  • Immuno-histochemistry
  • X-ray

Imaging tests may identify fluid around the heart and any visible pericardial tumors. However, procedures such as echocardiography can only distinguish fluid buildup. It cannot confirm the cause of the fluid buildup as mesothelioma.

Biopsies are an important step for an accurate diagnosis. In this procedure, a tissue sample from the affected area is analyzed under a microscope. A mesothelioma biopsy can determine cell type and help understand the stage of mesothelioma.

An accurate mesothelioma diagnosis is very important for pericardial mesothelioma. Worsening symptoms can have a severe impact on the body as the disease progresses.

As a result of the disease’s rarity and challenging diagnostic process, many pericardial mesothelioma patients aren’t diagnosed until autopsy. According to researchers, only about 10 – 25% of pericardial mesothelioma cases are diagnosed before a patient’s death.

Potential Misdiagnoses of Pericardial Mesothelioma

  • Various forms of pericarditis (inflammation of the tissue surrounding the heart)
  • Heart disease
  • Atrial myxoma (benign heart tumor)
  • Heart failure
  • Cardiomyopathy (heart muscle disease)
  • Tuberculosis

Misdiagnosis can lead to ineffective treatments and wasted time. To prevent misdiagnosis, patients may want to seek a second opinion to confirm their diagnoses.

05. Pericardial Mesothelioma Treatment

How Is Pericardial Mesothelioma Treated?

Pericardial mesothelioma may be treated with standard mesothelioma treatments, such as surgery and chemotherapy. Treatment options may vary based on disease stage, symptom severity, patient age and overall patient health.

Treatment options for pericardial mesothelioma include:

  • Surgical removal of tumors
  • Radiation
  • Pericardiectomy: Full or partial removal of the heart lining
  • Pericardiocentesis: Draining excess fluid around the heart with a needle
  • Pericardial window: Draining excess fluid by cutting a hole in the pericardium
  • Chemotherapy: Anti-cancer drugs, most often platinum-based (cisplatin or carboplatin) and pemetrexed

Most pericardial mesothelioma researchers urge a multimodal approach to treatment. Multimodal treatment plans combine two or more therapy types. For pericardial mesothelioma, patients treated with surgery and chemotherapy have had the best outcomes.

Treatment plans depend largely on the stage of the disease and the aim of the treatment. Currently, there are no curative options for this disease.

However, surgical tumor removal and chemotherapy may maximize mesothelioma patient’s life expectancy.

For late-stage diagnoses, treatment may be limited to palliative options. Palliative treatments aim to reduce symptoms and improve patient quality of life.

06. Pericardial Mesothelioma Prognosis

What Is the Prognosis for Pericardial Mesothelioma?

The prognosis for pericardial mesothelioma is generally poor. The life expectancy for peritoneal mesothelioma is about six months. However, some patients have lived much longer.

  • Researchers compared the survival periods of patients who received a single treatment versus those with multimodal treatment. Those with a single form had a median survival of 4.5 months, versus 16 months for the multimodal patients.
  • One case report documented a 54-year-old woman who has survived more than 4 years post-treatment and recurrence. Both times, doctors treated her cancer with a combination of chemotherapy and surgical resection.

The rarity of pericardial mesothelioma poses a problem for researchers. For instance, clinical trials for pericardial mesothelioma are difficult to execute because there are so few cases.

Newer therapies used on other forms of mesothelioma may have similar effects on pericardial mesothelioma. For example, immunotherapy has proven to be effective in treating other types of mesothelioma.

However, further research is needed to understand the intricacies of pericardial mesothelioma.

What Is the Life Expectancy of a Person With Mesothelioma?

Mesothelioma life expectancy varies due to location and cell type.

  • Average life expectancy for pleural mesothelioma is about 18 months
  • Average life expectancy for peritoneal mesothelioma ranges from two to six years
  • Average life expectancy for pericardial mesothelioma is six months