Pericardial Mesothelioma

Overview of Pericardial Mesothelioma

Pericardial mesothelioma represents approximately 1% of all diagnosed mesothelioma cases. With an estimated 3,000 cases of mesothelioma diagnosed in the United States every year, roughly 30 cases of pericardial mesothelioma are identified annually.

The prognosis for pericardial mesothelioma remains poor, with average survival of only a few months. While scientists are investigating the precise mechanisms of how the tumor spreads, the proximity to the heart can explain the poor survival outcomes.

In this same study, the median age of diagnosis is 46 years, notably younger than the median ages for pleural mesothelioma (approximately 70 years) and peritoneal mesothelioma (approximately 60 years). In a separate study of pericardial mesothelioma cases, the median age at diagnosis was 55 years old and 61% of the patients were men.

What Causes Pericardial Mesothelioma?

Asbestos exposure is the only cause of mesothelioma. When asbestos-containing materials are disturbed, microscopic fibers are released into the air. These fibers may be inhaled or ingested, subsequently becoming lodged in the linings of the lungs, abdomen or heart.

The pericardium, which is composed of mesothelial cells, may become affected when asbestos fibers migrate through the bloodstream or lymphatic system. Once lodged in the pericardial lining, the fibers can provoke chronic inflammation and genetic mutations in mesothelial cells, leading to uncontrolled cellular multiplication. When the normal cell death process does not work properly, abnormal cells accumulate and form a tumor.

Without medical intervention, pericardial mesothelioma tumors can spread to the heart and eventually to other vital organs, resulting in advanced-stage disease.

What Is the Pericardium?

The pericardium is the thin, protective sac that surrounds the heart and the major blood vessels connected to it. The pericardium consists of two layers of tissue with a small amount of fluid between them, which helps reduce friction as the heart beats.

The pericardium has several functions:

• Protects the heart and the surrounding blood vessels from outside pressure
• Provides lubrication to reduce friction between the heart and surrounding tissues
• Anchors the heart in the chest cavity
• Protects the heart from expanding too much or filling with too much blood
• Provides a barrier to protect the heart from infection

The vessels that enter or leave the heart are also protected by the pericardium. These include the:

• Aorta
• Pulmonary artery
• Pulmonary veins
• Superior vena cava
• Inferior vena cava

The pericardium has two layers:

• Fibrous pericardium – Outermost layer made of connective tissue that prevents the heart from overexpanding and protects the heart against infections
• Serous pericardium – Inner layer which produces fluid to lubricate the heart to reduce friction as it beats

The serous pericardium has two layers of tissue:

• Parietal pericardium – Outer layer attached to the fibrous pericardium
• Visceral pericardium – Inner layer covering the surface of the heart and blood vessel roots

The space between the parietal pericardium and the visceral pericardium is called the pericardial cavity. This space is filled with fluid, which reduces the friction between the two tissue layers to allow the heart to expand and contract.

Symptoms of Pericardial Mesothelioma

The symptoms of pericardial mesothelioma can be similar to other common heart conditions. The most common symptom is fluid buildup in the pericardium, which is the sac around the heart. This symptom is called pericardial effusions, and it can hinder normal cardiac function by adding pressure on the heart and preventing it from pumping normally.

Other pericardial mesothelioma symptoms include:

• Chest pain
• Shortness of breath
• Fatigue
• Irregular heartbeat
• Heart murmurs

How Is Pericardial Mesothelioma Diagnosed?

Diagnosing pericardial mesothelioma is often difficult and challenging for physicians. This is primarily because of how rare the disease is and the nonspecific nature of its symptoms, which often mimic those of more common heart conditions.

Symptoms like chest pain, fatigue, shortness of breath, and fluid buildup around the heart (pericardial effusion) do not immediately point to a rare cancer. This can lead to delayed recognition or a misdiagnosis.

Imaging tests like echocardiograms, CT scans, or MRIs may reveal abnormalities, but they are not definitive for diagnosing mesothelioma. Tissue biopsy remains the most accurate way to diagnose all types of mesothelioma, including pericardial mesothelioma.

Imaging Tests

Physicians rely on common imaging tests to determine the cause of a patient’s symptoms. These imaging tests include chest X-rays, CT scans, MRI scans and echocardiograms. The results of the tests provide the doctors with a detailed evaluation of the structures in the chest cavity along with the function of the heart.

Biopsies

While imaging tests can help reveal certain abnormalities and provide clues about the presence of disease, a tissue biopsy remains the definitive method for diagnosing pericardial mesothelioma.

During a biopsy, doctors remove tissue from the pericardium (the sac surrounding the heart) to examine it for cancerous cells. This procedure often requires a needle or other surgical methods to extract a sample, which is then analyzed in a laboratory using various staining techniques to confirm whether the cells are malignant.

Given the heart’s delicate and critical nature, performing a tissue biopsy can be risky, particularly when the tumor is located near vital heart structures. To reduce the potential for damage, some doctors prefer to use a less invasive approach: a fluid biopsy.

Pericardiocentesis not only helps diagnose mesothelioma but also provides some therapeutic relief by alleviating the pressure caused by fluid buildup, which improves symptoms like chest pain and difficulty breathing. However, while fluid analysis is helpful, it may not always provide a clear diagnosis, as the fluid sample extracted may not possess cancer cells even though disease exists. As a result, further testing may be necessary to confirm whether the patient has pericardial mesothelioma.

Treatment for Pericardial Mesothelioma

Treatment options are available for patients with pericardial mesothelioma. The two main options are surgery and chemotherapy. While immunotherapy is used for other types of cancer, including other types of mesothelioma, there has not been enough information about its use for it to be offered as a standard of care for pericardial mesothelioma.

Patients can also receive palliative therapies to improve their quality of life and provide comfort for their symptoms.

Since pericardial mesothelioma forms in the lining of the heart, options are limited. The heart is a vital organ, so any damage to it can be life-threatening. For this reason, doctors do not recommend radiation for pericardial mesothelioma.

Doctors must also be very selective of which patients they recommend for pericardial mesothelioma surgery. Any surgery involving the heart can be life-threatening, so doctors must evaluate patients carefully.

Surgery remains a limited treatment option for pericardial mesothelioma. Resection is generally reserved for patients in good health with localized disease, which means disease that has not spread to other parts of the body. Diagnosing pericardial mesothelioma in early stages is rare, which is why surgery is so uncommon.

The top surgical procedure for pericardial mesothelioma is a pericardiectomy, which requires removing part or all of the pericardium (the thin lining that surrounds and protects the heart). This lining is where pericardial mesothelioma originates before potentially spreading to nearby tissues.

In addition to removing the affected pericardium, surgeons may remove surrounding cancerous tissues, provided it can be done without endangering critical cardiac structures. The goal is to remove as much of the visible tumor as possible – to improve their long-term survival – without risking the patient’s safety.

Other studies echo these findings, noting that surgery may offer symptom relief for pericardial mesothelioma patients. Surgery can help reduce pericardial effusions (fluid accumulation around the heart) and help prevent cardiac tamponade, which is the result of pressure placed on the heart from the pericardial effusions.

Reported median survival after surgery ranges from 6-10 months. Outcomes vary depending on tumor stage, patient health, and multimodal treatment strategies.

Because of the heart’s sensitivity and the proximity of the tumor to critical cardiac structures, pericardial surgery carries considerable risk. It is usually performed in high-volume centers by experienced cardiothoracic surgeons.

Chemotherapy is a standard treatment option for pericardial mesothelioma, particularly in cases where the patient is not healthy enough for surgery or the disease has spread beyond the pericardium. This treatment sends cancer-killing drugs into the patient’s body (usually through the bloodstream).

The most common chemotherapy drugs for pericardial mesothelioma are pemetrexed and cisplatin. The combination of both is approved by the U.S. Food and Drug Administration (FDA) for pleural mesothelioma and often applied as an off-label treatment for pericardial mesothelioma due to the lack of formal clinical trials for this rare type.

Pemetrexed inhibits key enzymes involved in DNA formation. Cisplatin is a platinum-based compound that interferes with the cancer cells’ DNA, causing cell death.

Chemotherapy can lead to side effects, such as:

• Nausea
• Vomiting
• Fatigue
• Weight loss
• Body aches

More serious side effects include:

• Anemia – Low red blood count
• Neutropenia – Low white blood cell counts (resulting in increased risk of infection)
• Mouth sores and digestive disturbances
• Skin rashes
• Organ toxicity – Damage to the heart or liver

The Journal of Clinical Oncology reported that 39% of pericardial patients received chemotherapy.

Immunotherapy is an emerging form of cancer treatment that harnesses the body’s immune system to recognize and attack cancer cells. While immunotherapy has transformed treatment options for several cancers – including pleural mesothelioma – it remains largely untested for pericardial mesothelioma due to the extreme rarity of the disease.

The main barrier is pericardial mesothelioma accounts for less than 1% of all mesothelioma cases, with fewer than 200 reported in medical literature. This makes it extraordinarily difficult to enroll enough patients for meaningful clinical research, including trials for immunotherapy agents.

Despite the lack of direct evidence, some researchers speculate that immunotherapy may eventually have a role in the treatment of pericardial mesothelioma. The hope is as immunotherapy advances, compassionate use or individualized access might offer future options for certain patients, particularly those who have exhausted all other standard treatments.

Radiation , which uses high-energy beams such as X-rays or proton beams to destroy cancer cells, is not a standard treatment for pericardial mesothelioma. The primary reason is the anatomical location of the disease – tumors develop in the pericardium (the thin sac around the heart). Delivering effective doses of radiation to this area poses a high risk of radiation-induced heart damage, including cardiomyopathy (weakened heart muscle), pericarditis (inflammation of the pericardium), or coronary artery injury.

One of the key concerns with radiation is the potential for radiation “spray” or scatter, where surrounding healthy tissues receive unintended radiation exposure. While radiation may eventually play a limited role in the treatment of pericardial mesothelioma, most oncologists avoid radiation unless benefits clearly outweigh the risks.

According to the study in the Journal of Clinical Oncology, approximately 8% of patients with pericardial mesothelioma received radiation.

Since pericardial mesothelioma is typically diagnosed at an advanced stage – often after the disease has already compromised cardiac function or spread – palliative care plays a central role. The primary goal of palliative therapies is not to cure the disease but to relieve symptoms, reduce discomfort, and maintain the best possible quality of life for patients.

One of the most common and effective palliative procedures is pericardiocentesis, which is a minimally invasive surgery that drains excess fluid from the pericardial sac. Pericardial effusion – fluid buildup between the heart and its outer lining – is a frequent complication of this cancer and can lead to cardiac tamponade, which is a life-threatening condition where the heart is compressed and unable to function properly. Removing this fluid can significantly improve cardiac output, reduce shortness of breath, and alleviate chest pain.

In cases where fluid continues to accumulate, a more permanent solution may be considered, such as:

• Pericardial window surgery – Creates a small hole in the pericardium to allow continuous drainage of fluid into the chest cavity
• Indwelling catheter placement – Allows for at-home fluid drainage to manage recurring pericardial effusions

Additional palliative treatments may include:

• Pain management with medications such as opioids, NSAIDs or nerve blockers
• Anti-inflammatory drugs or corticosteroids to reduce tumor-related swelling
• Oxygen therapy for patients experiencing severe shortness of breath
• Nutritional support and counseling to help address weight loss and fatigue
• Emotional and psychological support, including counseling or hospice services

Since the disease is aggressive and prognosis is generally poor, a multidisciplinary team – oncologists, cardiologists, palliative care specialists, social workers and others – is often involved in helping patients and families make care decisions. Palliative care is a critical pathway for ensuring patients maintain comfort and dignity.

Pericardial Mesothelioma Prognosis

The prognosis for mesothelioma remains poor due to several significant challenges in diagnosing and treating the disease. The tumors develop in close proximity to the heart – a vital and delicate organ – which limits surgical options and makes aggressive treatment risky. Additionally, the symptoms – such as chest pain, fatigue and shortness of breath – are easily mistaken for other cardiac or respiratory conditions, contributing to delayed or missed diagnoses.

Dr. Raphael Bueno, a renowned thoracic surgeon and mesothelioma specialist at Brigham and Women’s Hospital, contributed to a comprehensive review published in the Annals of Thoracic Surgery. The study emphasized that prognosis is often discouraging in pericardial mesothelioma, citing the limited responsiveness to chemotherapy and the rarity of the disease as major barriers to improving outcomes. The review highlighted that even with multimodal therapy, including surgery and systemic treatments, long-term survival is uncommon.

Anyone diagnosed with mesothelioma and wants to learn how others have outlived their prognosis can get our free Survivors Guide book. We will deliver it directly to you at no cost.

Compensation for Pericardial Mesothelioma

Researchers are continuing to evaluate how pericardial mesothelioma tumors form, but the cancer is undoubtedly linked to asbestos exposure. Asbestos is the only proven cause of all types of mesothelioma, and the companies behind the mining, manufacturing, and selling of asbestos exposed people to this deadly mineral. They should be held accountable in the courts.

Expert mesothelioma lawyers can help you receive asbestos compensation for you or a loved one’s pericardial mesothelioma. While this cancer is rare, there are examples of patients taking asbestos companies to court and receiving deserved payments to recoup lost wages, medical bills and more.

There are two methods of receiving compensation for pericardial mesothelioma. Most patients file claims with asbestos trust funds, which are bank accounts with money set aside by bankrupt asbestos companies. Some patients file mesothelioma lawsuits if the at-fault companies are still in business.

Look for a lawyer with experience handling mesothelioma cases. Mesothelioma Guide has tips on how to find a mesothelioma lawyer so you get the best possible legal representation and guidance.

Lawsuit Against Johnson & Johnson for Pericardial Mesothelioma

In 2023, a landmark legal case drew national attention when a pericardial mesothelioma patient filed a lawsuit against Johnson & Johnson, alleging asbestos exposure from the company’s talc-based Johnson’s Baby Powder. This case is particularly significant due to the rarity of pericardial mesothelioma and the unusually young age of the plaintiff – just 24 years old at the time of diagnosis.

According to court filings, the plaintiff’s family regularly used Johnson & Johnson’s Baby Powder during his infancy. The product, which historically contained talc, a naturally occurring mineral. Talc has been under scrutiny for years due to asbestos contamination.

Deposits of talc and asbestos are frequently found in close proximity to one another, and without rigorous purification, talc products can become contaminated during mining and processing. Asbestos is a known carcinogen, and exposure to asbestos has been linked to mesothelioma.

In this case, the plaintiff’s legal team argued that early and repeated exposure to asbestos-contaminated talcum powder during infancy directly contributed to the development of pericardial mesothelioma decades later. Expert testimony and internal company documents were presented as evidence of Johnson & Johnson’s alleged knowledge of asbestos risks in its products – and of their failure to provide adequate warnings to consumers.

This case adds to the growing list of litigation involving asbestos exposure and consumer talc products, which has already resulted in billions of dollars in claims against Johnson & Johnson. While most mesothelioma cases associated with talc involve pleural or peritoneal mesothelioma, this verdict of a pericardial mesothelioma case underscores the far-reaching implications of contaminated consumer goods.

Help for Patients with Pericardial Mesothelioma

If you’d like more information about pericardial mesothelioma, you can speak with our patient advocates. Registered nurse Karen Ritter works with mesothelioma patients and families to find the best treatment options for their unique diagnosis, along with options for compensation and support groups.

Email Karen at karen@mesotheliomaguide.com. You can also request a free Complete Guide to Mesothelioma book for an in-depth explanation of pericardial mesothelioma and the other types of mesothelioma, the treatment options you could consider, and more.