What Is Pericardial Mesothelioma?

Pericardial mesothelioma is a rare cancer that affects the heart’s lining, the pericardium. Less than 1% of all types of mesothelioma are pericardial.

Asbestos exposure causes mesothelioma cancer. The link between asbestos and pericardial cancer is still under investigation. Researchers are working to understand how asbestos fibers reach the heart. 

Pericardial Mesothelioma diagram
Diagram shows pericardial mesothelioma tumors forming on the smooth lining around the heart.

This cancer usually affects people between 50 and 70 years of age. It also occurs in men more often than women. Symptoms appear at a later stage and can mimic other heart disorders. These can include chest pain, fatigue and shortness of breath. Diagnosis is challenging due to these similar symptoms.

Pericardial treatment options include surgery and chemotherapy. Some patients elect no treatment. Only a few patients receive radiation therapy. This treatment carries a higher risk near the heart.

Fewer than 150 cases of pericardial mesothelioma exist in medical literature. A 2023 research report notes that median survival is about 2 to 6 months. Survival rates are low, but some patients can live years after surgery or chemotherapy.

What Are the Symptoms of Pericardial Mesothelioma?

Common pericardial symptoms include difficulty breathing, chest pain and heart palpitations. Fluid buildup around the heart and thickening of the pericardial layers cause symptoms.

Most pericardial mesothelioma patients experience no symptoms at first. This fact contributes to a late-stage diagnosis. The signs of pericardial mesothelioma can also resemble those of other heart conditions. This similarity makes it difficult to diagnose. 

Recommended tests include an X-ray, CT scan or echocardiogram (heart ultrasound).

Symptoms of Pericardial Mesothelioma

  • Chest pain
  • Cough
  • Difficulty breathing, even when at rest (dyspnea)
  • Fatigue
  • Fever
  • Heart palpitations or irregular heartbeat (arrhythmia)
  • Murmurs
  • Night sweats
  • Pericardial effusion
  • Shortness of breath when lying flat

If you are experiencing symptoms, schedule an appointment with your doctor immediately. Screening tests can reveal the underlying cause of these health problems.

How Is Pericardial Mesothelioma Diagnosed?

A physical examination and imaging tests can diagnose the location of pericardial mesothelioma tumors. A biopsy can confirm whether they are cancerous. 

What is Pericardial Effusion?
Diagram shows the area of the pericardium where excess fluid builds up.

Doctors will also assess your symptoms, medical history and current medical condition. They use this information, along with biopsy results, to determine treatment.

  • Physical exams can uncover signs and symptoms of pericardial mesothelioma. Findings during this exam will lead to imaging tests. Unfortunately, most cases are discovered during an autopsy. Doctors diagnose about 10%-20% of cases before a patient dies.
  • An echocardiogram is one of the first diagnostic tests you will receive. This test is an ultrasound of your heart that can reveal excess fluid. It helps doctors see the size, shape and function of your heart. The scan is noninvasive and uses sound waves.
  • Imaging tests such as computed tomography (CT) and magnetic resonance imaging (MRI) scans help detect pericardial tumors. This makes them a preferred diagnostic tool for determining whether a biopsy is necessary.
  • A biopsy will test the makeup of abnormal growths. Doctors will surgically remove fluid or tissue from the mass. A pathologist views the cells under a microscope and confirms whether you have mesothelioma and the specific subtype.

Pericardial tumors generally are not localized. They tend to cover most of the heart. This cancer type accounts for approximately half of all pericardial tumors.

Seek a Second Opinion to Avoid Misdiagnosis

It’s vital to meet with a specialist. They must confirm a pericardial mesothelioma diagnosis. A mesothelioma expert will know more about this rare cancer. They can ensure an accurate diagnosis and prompt treatment.

Common Pericardial Mesothelioma Misdiagnoses

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

If you have been diagnosed with any of these conditions, always seek a specialist. Mesothelioma oncologists and cardiothoracic surgeons can diagnose and treat pericardial mesothelioma. These experts can recommend new therapies and clinical trials that may improve survival.

What Are the Treatment Options for Pericardial Mesothelioma?

Mesothelioma treatment for pericardial mesothelioma includes surgery and chemotherapy. The heart lining rests close to the heart and limits treatment options.

Surgery is the most effective treatment for this cancer, but more than half of patients are not eligible. In rare cases of an early diagnosis, surgery may be able to remove small, localized tumors. The primary surgical treatment options for pericardial mesothelioma are pericardiectomy and tumor removal.

Chemotherapy and palliative treatment, such as fine needle aspiration, can ease symptoms and slow growth. Radiation therapy is considered minimally beneficial for this rare cancer. It is risky to administer without harming the heart.

Pericardiectomy or Tumor Removal

Patients who are good candidates for surgery may undergo a pericardiectomy or tumor removal. A pericardiectomy removes part or all of the pericardium. This surgery relieves pressure and minimizes fluid buildup.

Tumor removal, or tumor resection, removes cancer without removing the pericardium. A 2017 review reported more prolonged survival with tumor removal than a pericardiectomy.

Chemotherapy

The benefits of chemotherapy are minimal for most patients with pericardial mesothelioma. The chemotherapy drugs pemetrexed and cisplatin may improve survival. Gemcitabine has produced mixed results.

A handful of cases have responded well to chemotherapy. One woman lived longer than two years thanks to the chemo drugs cisplatin, gemcitabine and vinorelbine.

Palliative Treatment

Fluid buildup in the pericardium is the primary cause of symptoms. Palliative treatment options aim to minimize pain and reduce symptoms. This care can improve the quality of life and make patients more comfortable.

We speak with 1-3 pericardial mesothelioma patients or their loved ones each year. This is an extremely rare diagnosis that most doctors have not seen in their careers. With the help of our Doctor Match program, we are able to connect these patients to world-renowned experts to explore treating this rare diagnosis.

A pericardiocentesis removes excess fluid to relieve pain and pressure around the heart. Pain medication can also reduce symptoms and improve comfort. 

What Is the Average Prognosis?

The prognosis for pericardial mesothelioma is poor compared to peritoneal or pleural mesothelioma. Nearly 50% to 60% of pericardial patients die within six months of diagnosis. However, this is not the case for everyone. About 26% of patients live for one year or more. A 2024 case report says survival time is around 2.5 years. The 5-year survival rate for pericardial mesothelioma is 9%. 

Hope for improved mesothelioma life expectancy exists for some patients because researchers have cited positive results from surgery. Research also shows minor, less impactful benefits from chemotherapy, except for one case. A 47-year-old woman lived two years after chemotherapy with cisplatin, gemcitabine and vinorelbine.

A 1995 case report of a 27-year-old woman who underwent surgery and radiation therapy for pericardial mesothelioma states that she had no evidence of recurrence during 28 years of follow-up.

Surgery to remove part of the heart’s lining combined with radiation therapy improved survival in two patients in the late 1960s and early 1970s. One patient lived a year after treatment, and another was alive five years after treatment.

Dr. Jacques Fontaine and Dr. Andrea Wolf
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How You Can Cope With Pericardial Mesothelioma

Several informational resources are available for patients diagnosed with pericardial mesothelioma. Available support resources include:

  • Community Organizations. Many community organizations offer support and resources to patients with cancer. These associations may provide support groups, educational resources and financial help.
  • Counseling. Counseling can be helpful for patients with pericardial mesothelioma and their families. Professional counselors can provide emotional support. They can also offer guidance on how to manage stress and anxiety.
  • Hospital Services. Many hospitals have specialized oncology departments. These units offer pain management, nutrition counseling and physical therapy.

These resources can help patients better understand their condition and treatment options. They can also help them cope with the disease’s physical and emotional challenges.

Pericardial Mesothelioma Coping Strategies

Coping with pericardial mesothelioma can be challenging. Effective coping strategies can manage symptoms and improve quality of life. Some helpful tips include:

  • Practice self-care. Exercise, healthy eating and quality sleep can help manage symptoms. They’ll also improve general well-being and allow you to tolerate more treatments.
  • Stay organized. Keep track of appointments, medications and symptoms. This can help you feel more in control of your condition. Use a journal or planner to stay organized.
  • Seek emotional support. It’s vital to ask for help from loved ones, friends or professional counselors. Joining a support group can also be helpful. You can connect with others who are going through similar experiences.

Pericardial mesothelioma can be challenging, but many resources are available. Take advantage of these resources. Practice self-care, stay organized and seek emotional support as needed.

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