01. What Is Pericardial Mesothelioma?
What Is Pericardial Mesothelioma?
Pericardial mesothelioma is an extremely rare type of cancer that forms in the lining around the heart, called the pericardium. This type accounts for 1% or less of all mesothelioma diagnoses. Signs of pericardial mesothelioma include chest pain, fatigue and heart problems.
In medical literature, only a few hundred cases of pericardial mesothelioma have been reported. With so few cases, research is limited. However, researchers’ understanding of life expectancy and the most effective treatment continues to evolve.
02. Pericardial Mesothelioma Causes
What Causes Pericardial Mesothelioma?
Research on what causes this disease is limited because so few cases of pericardial mesothelioma have been reported. However, researchers have determined potential risk factors for pericardial mesothelioma. A risk factor is something that increases a person’s chances of developing a disease. It is not necessarily the cause of the disease.
Potential Pericardial Mesothelioma Risk Factors
- Erionite exposure
- Radiation exposure
- Simian virus 40 infection
- Thorotrast (a dangerous contrast agent historically used in certain medical scans)
Asbestos Exposure and Pericardial Mesothelioma
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.
Identifying the cause of pericardial mesothelioma can be difficult because isolating where the asbestos exposure happened is not always possible.
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. Patients may have symptoms of the disease at early stages because of the involvement of the heart. However, because pericardial mesothelioma is rare, it can be difficult for doctors to identify these symptoms as signs of mesothelioma.
Pericardial Mesothelioma Symptoms
Pericardial mesothelioma symptoms can be vague and easily attributed to other conditions. The following symptoms have been observed in pericardial mesothelioma patients:
- Cardiac tamponade
- Chest pain
- Dry cough
- Dysphagia (difficulty swallowing)
- Pericardial effusion
- Pericardial thickening
- Right shoulder pain
- Shortness of breath
- Swelling of the legs or lower extremities
The vagueness of pericardial mesothelioma symptoms creates 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:
- CT-guided liquid biopsy
- CT scan
- PET scan
Imaging tests may identify fluid around the heart (pericardial effusion) and any visible pericardial tumors. However, an echocardiography can only distinguish fluid buildup. It cannot confirm that the cause of the fluid buildup is mesothelioma. If an echocardiogram reveals fluid buildup, doctors may then conduct other procedures, like biopsies, to determine the cause.
Biopsies are an important step for an accurate diagnosis. In these procedures, a fluid or tissue sample is collected from the affected area. A technician then analyzes the sample 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
- Atrial myxoma
- Heart disease
- Heart failure
- Pleural empyema
- Various forms of pericarditis
In rare cases, pericardial mesothelioma may be masked by other conditions. In one case report, a patient had pleural empyema (a collection of pus in the lining around the lung). Empyema prevented doctors from finding the pericardial mesothelioma with imaging. Doctors found the tumor with exploratory surgery.
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.
What Are the Treatment Options for Pericardial Mesothelioma?
Treatment options for pericardial mesothelioma include chemotherapy, surgery and palliative procedures. In rare cases, doctors may also administer radiation therapy.
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.
Doctors may incorporate chemotherapy into pericardial mesothelioma treatment plans. Mesothelioma patients are commonly treated with systemic chemotherapy drugs. Standard mesothelioma chemotherapy drugs include pemetrexed, cisplatin and carboplatin.
Doctors may perform surgery to remove pericardial tumors and affected tissue. This can help remove cancer cells, slow tumor spread and relieve pressure on the heart and other organs. Pericardial mesothelioma patients may undergo a pericardiectomy. For this surgical procedure, doctors conduct a full or partial removal of the heart lining.
Surgery is a common component of multimodal treatment plans for pericardial mesothelioma.
Treatment plans depend largely on the stage of the disease and the aim of the treatment. Currently, there are no treatment options to cure this disease.
However, surgical tumor removal and chemotherapy may maximize a 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.
Palliative and Symptom Relief Procedures
Fluid buildup associated with pericardial mesothelioma, called pericardial effusion, can be very uncomfortable for patients. It may even cause heart troubles from increased pressure on the muscle.
There are two main ways of draining pericardial effusion fluid: pericardiocentesis and pericardial windows. In a pericardiocentesis procedure, doctors use a needle to drain excess fluid around the heart. In a pericardial window procedure, doctors cut a small hole in the pericardium to drain excess fluid.
06. Pericardial Mesothelioma Prognosis
What Is the Prognosis for Pericardial Mesothelioma?
The prognosis for pericardial mesothelioma is generally poor. The life expectancy for pericardial patients is about six months. However, some patients have lived much longer. Some pericardial mesothelioma patients have experienced prolonged survival:
- In one study, researchers compared the survival periods of pericardial mesothelioma 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. The most popular multimodal treatment in this study combined surgery and adjuvant chemotherapy.
- Another case report documented a 54-year-old woman who 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.
Ongoing research may lead to more effective treatment options. For now, pericardial mesothelioma is very rare and often has a less favorable prognosis than other forms of the cancer. Patients diagnosed with this unusual cancer should discuss it with a mesothelioma specialist. The doctor can explain treatment options and what to expect.
Resources for Pericardial Mesothelioma Patients
07. Common Questions
Common Questions About Pericardial Mesothelioma
How rare is pericardial mesothelioma?
- Pericardial mesothelioma is extremely rare, making up 1% or less of all mesothelioma diagnoses. Medical publications reported fewer than 500 cases between the early 1900s and early 2000s.
What is the life expectancy for pericardial mesothelioma?
- Average pericardial mesothelioma life expectancy is six months. However, patients can live longer with proper treatment, which often includes surgery and chemotherapy.