Pericardial Mesothelioma

Pericardial mesothelioma is a rare form of cancer that affects the lining of the heart (pericardium). It is the rarest of the three main types of mesothelioma, making up only 1% of all cases, and it has the lowest life expectancy — 6-12 months. Symptoms such as chest pain and coughing are often vague and may mimic signs of more common heart disorders, so most diagnoses are made after autopsy. However, new treatments may help patients live longer.

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What Is Pericardial Mesothelioma?

Pericardial mesothelioma is a rare kind of mesothelioma that forms in the sac-like lining surrounding the heart (pericardium).

Because of its exceptional rarity, how difficult it is to detect, and how close it is to the delicate heart, pericardial mesothelioma has the most dire prognosis of all mesothelioma types. However, treatment may help extend a patient’s life.

Quick Facts About Pericardial Mesothelioma

  • Pericardial mesothelioma is very rare, with around 350 cases reported.
  • Pericardial mesothelioma patients are diagnosed at a median age of 55, according to the 2019 literature review published in the journal Clinical Lung Cancer — a  far younger population than the median for other mesothelioma types.
  • The same review found that the median survival time of pericardial mesothelioma patients was 6 months.
  • As many as 80-90% of pericardial mesothelioma patients are only diagnosed after death, according to a 2013 study from the Journal of Cardiovascular and Thoracic Research.

Pericardial Mesothelioma Causes

Pericardial mesothelioma occurs when malignant (cancerous) tumors form in the protective lining around the heart.

What causes it is still unclear, but asbestos exposure is a likely cause in at least some cases. Unlike with other types of mesothelioma, most pericardial mesothelioma patients do not have a known history of asbestos exposure.

Still, patients with symptoms of this disease should share any asbestos exposure history with their doctors.

How Pericardial Mesothelioma Develops

Doctors know fairly little about how pericardial mesothelioma develops, especially in cases where patients have no known history of asbestos exposure.

Generally, mesothelioma develops decades after a patient is exposed to asbestos.

Asbestos fibers are so small and strong that they can remain in the body after being inhaled. Instead of being broken down or removed through coughing, they irritate and inflame the tissues lining the organs. They can also travel from the lung to other organs nearby organs.

In the case of pericardial mesothelioma, this irritation happens in the lining of the heart, eventually leading to mutations in cells and the growth of mesothelioma cancer cells.

Doctors are still not completely sure how the fibers find their way to the heart.

Who Is at Risk for Pericardial Mesothelioma?

Less than 1% of mesothelioma victims develop pericardial mesothelioma. With so few cases, it is difficult to predict who may be at risk for the disease.

A study published in the International Journal of Hygiene and Environmental Health found no strong link between pericardial mesothelioma and asbestos exposure.

However, someone may be at higher risk for pericardial mesothelioma if they are:

  • Male: About 60% of pericardial mesothelioma patients are men.
  • Exposed to asbestos: Other studies have found a link between pericardial mesothelioma and asbestos exposure, such as the 2011 case report Pericardial Mesothelioma and Asbestos Exposure authored by the Department of Preventive Medicine in Milan, Italy.
  • Older: Despite pericardial mesothelioma patients’ comparatively low median age at diagnosis (55), a 2017 study found that the rates of pericardial mesothelioma increased with age.

As medical professionals continue to review pericardial mesothelioma cases, more light will hopefully be shed on the risk factors and causes of this rare disease.

Pericardial Mesothelioma Symptoms

Pericardial mesothelioma symptoms are often vague and may mimic signs of more common heart disorders. In addition, symptoms may take 20-50 years to develop after asbestos exposure, making the link hard to detect — if it exists at all.

Symptoms of pericardial mesothelioma include:

  • Chest pain
  • Cough
  • Difficulty breathing (dyspnea)
  • Fatigue
  • Fever
  • Heart murmurs
  • Irregular heartbeat (arrhythmia)
  • Night sweats
  • Shortness of breath
  • Unexplained weight loss

Most symptoms are caused by fluid buildup in the lining of the heart (pericardial effusion) or by pericardial thickening.

Diagnoses are usually made when these symptoms begin to be explored.

Pericardial Mesothelioma Diagnosis

The sooner a patient makes a pericardial mesothelioma diagnosis, the better their overall outlook.

Unfortunately, between its rarity and vague symptoms, pericardial mesothelioma diagnoses are difficult to make. Most cases are found on autopsy after death.

The following help to diagnose pericardial mesothelioma.

Imaging Tests

Doctors first turn to imaging scans to detect fluid buildup around the heart and to determine if tumors are present.

While imaging tests cannot confirm a pericardial mesothelioma diagnosis, they are an important early step toward diagnosis.

Doctors may use the following imaging tests to make a pericardial mesothelioma diagnosis:

  • Computed tomography (CT) scan
  • Echocardiography
  • Magnetic resonance imaging (MRI)
  • Radionuclide imaging

Once the site of any fluid buildup, pericardial thickening, or potential tumors has been located through imaging, doctors are ready to collect tissue or fluid samples.

Biopsies

Biopsies are used to confirm a pericardial mesothelioma diagnosis. During a biopsy, a surgeon removes tissue from the pericardium and, a pathologist examines its cells under a microscope to determine if they are cancerous.

Getting a Second Opinion

The symptoms of pericardial mesothelioma resemble many other conditions, and the disease is so rare that most doctors may not even consider it when making a diagnosis.

Therefore, it is crucial to get a second opinion from a mesothelioma specialist — particularly one who has experience with pericardial mesothelioma.

Pericardial Mesothelioma Stages

Cancer is usually diagnosed by stage — the level of severity the disease has reached — but not enough is known about pericardial mesothelioma for doctors to break it down into the typical stages of mesothelioma.

Instead, pericardial mesothelioma is classified as advanced or localized.

Unfortunately, most patients are diagnosed during the advanced stage of the disease, contributing to pericardial mesothelioma’s poor prognosis.

Pericardial Mesothelioma Prognosis

A mesothelioma prognosis describes the general course the disease takes, including a patient’s chances for recovery.

Pericardial mesothelioma’s prognosis is poor, even compared to that of pleural and peritoneal mesothelioma. On average, 50-60% of pericardial mesothelioma patients do not live more than 6 months after diagnosis.

However, there are always exceptions to the average. For example, one pericardial mesothelioma patient who received a special 3-drug regimen of chemotherapy remained disease-free for 24 months after treatment.

Factors That Affect Prognosis

Not much is known about pericardial mesothelioma prognosis. However, many factors may affect the disease’s course.

Pericardial mesothelioma prognosis may be affected by:

In rare cases, patients have beaten the odds and survived more than a year after diagnosis.

Improving Pericardial Mesothelioma Prognosis

Pericardial mesothelioma patients may be able to take certain actions to help improve their prognosis.

To improve their pericardial mesothelioma prognosis, patients should:

  • Seek early diagnosis: The earlier patients are diagnosed, the more likely they are to be eligible for curative surgeries that may extend their lives by several months. This is especially important for pericardial mesothelioma patients, who often do not respond well to chemotherapy and radiology.
  • Maintain health: Generally, mesothelioma patients benefit from eating well and staying as active as possible. Such actions may boost their immune systems, decrease stress, and improve blood flow, improving overall health and recovery after treatment.
  • Manage stress: High stress levels contribute to worse overall patient health and may harm the heart — which is harmful for pericardial mesothelioma patients.

Pericardial Mesothelioma Treatment

Pericardial mesothelioma treatment options may be limited for most patients.

Because pericardial mesothelioma lies so close to the heart, it can be difficult to use aggressive treatments without causing too much damage to the heart itself.

In addition, pericardial mesothelioma is often diagnosed when it has already advanced considerably, making such patients ineligible for the most effective treatment method, surgery.

The primary treatment methods for pericardial mesothelioma are:

  • Chemotherapy
  • Palliative treatments
  • Pericardiectomy (tumor removal)

When possible, doctors attempt a multimodal treatment approach consisting of surgery, chemotherapy, radiotherapy, and/or immunotherapy.

Surgery

Surgery is often the most effective way to improve the lifespan of pericardial mesothelioma patients.

Most pericardial mesothelioma patients undergo 1 of 2 surgeries:

  • Pericardiectomy: This surgery involves the removal of tumors by removing part or all of the pericardium.
  • Tumor resection: During tumor resection, surgeons remove cancerous tumors without removing the pericardium.

A 2017 review published in Herz medical journal found that tumor resection patients lived longer than patients who had a pericardiectomy.

Chemotherapy

Overall, chemotherapy has not proven to be particularly effective in treating pericardial mesothelioma.

However, some patients have responded well to the chemotherapy drugs pemetrexed and cisplatin. The combination of cisplatin, gemcitabine, and vinorelbine have also shown promise in slowing the progression of the disease.

When possible, chemotherapy is used in addition to surgery to help slow the regrowth of tumors.

Radiation

Specialists rarely prescribe radiation therapy as part of a pericardial mesothelioma treatment plan.

Although there have been some success stories, radiation generally has had little effect on pericardial mesothelioma. More importantly, it can be dangerous to use near the heart.

Clinical Trials

Clinical trials are a type of research that studies a new treatment’s safety and effectiveness. In addition to advancing new treatments for pericardial mesothelioma, they may also give hope to current patients who may have few treatment options.

Although clinical trials for pericardial mesothelioma are rare, pleural and peritoneal mesothelioma trials also play a large role in finding new approaches for the former’s treatment.

Emerging Treatment Options

Some new cancer treatments have shown success with pericardial mesothelioma patients.

Promising new treatment options include:

  • Anti-angiogenesis: During this treatment, scientists use special drugs to block the growth of blood vessels in cancerous tumors rather than blocking cancer cells, themselves. Without extra blood, tumors are unable to grow beyond a few millimeters.
  • Gene therapy: Gene therapy is the process of changing, removing, or adding genes to a patient in order to fight or prevent a disease. Gene therapy is being tested to fight cancer in several ways.
  • Immunotherapy: This treatment involves training the body’s own immune system to effectively target and kill cancer cells.
  • Photodynamic therapy: This therapy makes cancerous tissue photosensitive with a specious drug and then uses a laser light to kill the cancer.

These new treatment options are currently being studied in clinical trials.

Palliative Treatment

The goal of palliative treatment is to reduce pain and discomfort rather than extend the life of a patient. Most treatments for pericardial mesothelioma are palliative because the disease is often diagnosed late after the cancer has spread considerably.

Palliative treatment for pericardial mesothelioma may take the form of:

  • Chemotherapy: Chemotherapy can slow the growth of tumors, which may, in turn, help prevent symptoms.
  • Medication: A doctor may prescribe pain medication to help patients deal with their symptoms.
  • Pericardiocentesis: During a pericardiocentesis, a surgeon inserts a small catheter into the heart lining and drains fluid buildup to ease the pressure caused by pericardial effusions.
  • Pericardiectomy: Removing the lining of the heart eases inflammation-caused pressure and stops some painful chronic symptoms such as fluid buildup around the heart.

Alternative therapies such as acupuncture, stress reduction practices, and relaxation techniques may also help patients cope with the symptoms of pericardial mesothelioma.

Top Pericardial Mesothelioma Specialists

Working with an experienced mesothelioma doctor is critical to receiving the best care for pericardial mesothelioma.

Pericardial mesothelioma specialists may help:

  • Give the most accurate diagnosis
  • Give advice on the best ways to improve a patient’s prognosis
  • Perform surgeries and other treatments that require high skill and experience
  • Provide the best treatment results
  • Refer patients to other specialists that may better meet their needs

Unfortunately, because pericardial mesothelioma cancer is so rare, few specialists exist. Some pleural mesothelioma specialists also have experience with pericardial mesothelioma.

Pericardial Mesothelioma Cancer Centers

Mesothelioma cancer centers specialize in studying and treating mesothelioma, hiring specialists capable of tailoring treatment toward each patient’s needs.

Many mesothelioma cancer centers treat pericardial mesothelioma along with more common forms of the disease.

Pericardial mesothelioma cancer centers include:

  • Brigham & Women’s Hospital
  • H. Lee Moffitt Cancer Center
  • Memorial Sloan Kettering Cancer Center
  • UT Southwestern Medical Center

Patients should ask a mesothelioma specialist if their center is right for them or if they can recommend a center that may be more suitable for pericardial mesothelioma patients.

Cost of Pericardial Mesothelioma Treatment

The average estimated cost for mesothelioma treatment totals around $500,000.

In addition, patients may have a number of unexpected costs — some of which may not be covered by insurance.

The cost of mesothelioma treatment may vary according to:

  • Caregiving needs
  • Healthcare providers
  • Individual treatment plan
  • Insurance coverage
  • Lodging needs
  • Lost income
  • Travel requirements
  • Veteran status

Thankfully, there are a number of ways that pericardial mesothelioma patients and their families can minimize these expenses.

Support Options for Pericardial Mesothelioma

Treatment for pericardial mesothelioma is often expensive, but there are several ways to help cover the associated expenses. Some, such as health insurance, mainly cover the cost of treatment, while others may be more flexible.

The following options may help mesothelioma victims cover treatment costs:

  • Asbestos trust funds: The U.S. government ordered bankrupt asbestos companies to contribute to these trust funds. They cover the costs of asbestos-related diseases employees suffered due to the wrongful actions of the companies.
  • Department of Veterans Affairs (VA) benefits: The VA not only provides coverage for medical treatment to qualified veterans — they have programs to help with the costs of travel, lodging, caregiving, end-of-life, and other needs.
  • Medicare and Medicaid: These government healthcare services cover mainly low-income individuals and offer coverage for mesothelioma of all types.
  • Private insurance: Most private health insurance policies cover the cost of mesothelioma treatment, although out-of-pocket costs may vary widely between policies.

Despite the challenges surrounding this incredibly rare cancer, pericardial mesothelioma patients and their families do have options for both treatment and funding.

Mesothelioma specialists continue to improve their understanding of the disease and its treatment every day — a fact reflected in the steadily increasing survival rates of all mesothelioma patients.

Mesothelioma Support Team
Reviewed by:Mark Levin, MD

Certified Oncologist and Hematologist

  • Fact-Checked
  • Editor

Mark Levin, MD has nearly 30 years of experience in academic and community hematology and oncology. In addition to serving as Chief or Director at four different teaching institutions throughout his life, he is also still a practicing clinician, has taught and designed formal education programs, and has authored numerous publications in various fields related to hematology and oncology.

Dr. Mark Levin is an independently paid medical reviewer.

Stephanie KiddWritten by:

Editor-in-Chief

Stephanie Kidd works tirelessly as a dedicated advocate for the vulnerable and underrepresented. Stephanie worked as a copywriter for an agency whose focus was communicating safety procedures on construction work sites. With her extensive background in victim advocacy and a dedication to seeing justice done, Stephanie works hard to ensure that all online content is reliable, truthful and helpful.

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