Written By: Karen Selby, RN,
Last modified: April 5, 2021

What is Pericardial Mesothelioma?

Pericardial mesothelioma is a rare type of cancer that affects the lining of the heart. To date, less than 150 cases of pericardial mesothelioma are described in medical literature — that’s 1 percent of all known diagnosed mesotheliomas.

Almost all mesotheliomas can be traced to asbestos exposure, but medical researchers continue to study the link between asbestos and cancer of the pericardium. About 25 percent of people with this disease have a history of asbestos exposure. Studies are clear on how inhaled microscopic fibers reach the lungs but less clear on how the fibers may reach the heart.

This form of cancer is most often diagnosed in people between the ages of 50 and 70, with the median age being 55. According to a 2017 study published in the Journal of Clinical Oncology, approximately 60 percent of cases occur in men.

Like the other types of mesothelioma, the disease 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 heart disorders.

Pericardial treatment options include surgery, chemotherapy, palliative treatments to control symptoms and rarely radiation therapy. Surgery is the most common treatment with about 46 percent of patients electing surgery. About 37 percent receive chemotherapy and only 8 percent receive radiation therapy because it is risky to apply near the heart. Nearly 25 percent of patients elect no treatment.

Average survival among people with pericardial mesothelioma is six to 10 months. Some people have lived for years with the cancer after undergoing surgery or chemotherapy.

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, CT scan or echocardiogram (ultrasound of the heart).

The following symptoms may indicate pericardial mesothelioma:

  • Difficulty breathing, even when at rest (dyspnea)
  • Heart palpitations or irregular heartbeat (arrhythmia)]
  • 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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What Causes Pericardial Mesothelioma?

The primary cause of pericardial mesothelioma remains a mystery. The causal relationship between asbestos exposure and this type of mesothelioma is not fully understood. Researchers confirm the pleural and peritoneal types are primarily caused by exposure to asbestos, yet the causes of the pericardial type are less definitive.

A 2017 review of medical literature on pericardial mesothelioma reported asbestos exposure in 25 percent of cases. Another 2017 study published in the Annals of Epidemiology reported the majority of patients with pericardial mesothelioma have no history of asbestos exposure.

How Is Pericardial Mesothelioma Diagnosed?

To diagnose pericardial mesothelioma, doctors make an overall 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.

Diagram showing pericardial mesothelioma affecting the heart
Pericardial mesothelioma is thought to occur when asbestos lodges itself around the heart.

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 is working overall.

Although an echocardiogram can reveal fluid buildup around the heart and help guide a pericardiocentesis, which is 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.

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.

Physical Examination

Unfortunately most cases of pericardial mesothelioma are only found at autopsy. Nonetheless, about 10-20 percent of these cases are diagnosed before a patient dies. Some of the challenges with early and accurate diagnosis of the pericardial type include the small number of people with the disease and the late presentation of symptoms.

Imaging Tests

Computed tomography (CT) scans are effective for detecting pericardial tumors, making them a preferred diagnostic tool. Magnetic resonance imaging (MRI) can also yield successful results in identifying the cancer.

Radionuclide imaging is a detection method involving the injection of gallium, 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.

Biopsy

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. According to a 2016 study in the Journal of Cardiac Failure, fluid biopsies often turn up cancer free even when pericardial tumors are present. A tissue sample of the tumor itself is usually required to confirm pericardial mesothelioma.

Seek a Second Opinion to Avoid Misdiagnosis

Because pericardial mesothelioma symptoms such as 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 the diagnosis. Working with a specialist may get 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?

    Mesothelioma treatment options are limited because the heart lining rests so closely to the heart itself that most therapies can easily damage this delicate organ. Slightly more than half of 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.

    The primary treatment options for pericardial mesothelioma are pericardiectomy or tumor removal, chemotherapy and palliative treatment such as fine needle aspiration. Radiation therapy is considered minimally effective for this rare cancer and is risky to administer without harming the heart.

    Pericardiectomy or Tumor Removal

    Patients whose doctors decide they are good candidates for surgery are treated with a pericardiectomy or tumor removal.

    A pericardiectomy is 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.

    Tumor removal, also called tumor resection, can involve removal of the cancer without removing the pericardium. A 2017 review reported longer survival in patients who underwent tumor removal than patients who had a pericardiectomy.

    Chemotherapy

    The benefits of chemotherapy are minimal for most patients with pericardial mesothelioma. The chemotherapy drugs pemetrexed and cisplatin have shown a survival benefit, and gemcitabine has produced mixed results. A handful of cases have responded well to chemotherapy. One woman lived longer than two years thanks to a combination of the chemotherapy drugs cisplatin, gemcitabine and vinorelbine. Pembrolizumab, a new checkpoint inhibitor form of immunotherapy, has shown some benefit in cases of pleural mesothelioma. It may have some utility in this disease as well.

    Palliative Treatment

    Palliative treatment options aim to minimize pain and reduce 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. A pericardiocentesis removes excess fluid from the pericardium to relieve pressure around the heart.

    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. Smaller, less impactful benefits have been demonstrated from chemotherapy, except for one case in which a 47-year-old woman was living 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.

    A 1992 case report of a 12-year old boy with pericardial mesothelioma who underwent surgery and chemotherapy says that he remained free of symptoms and showed no sign of disease one year later.

    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.


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