Pericardial mesothelioma is a cancer of the lining that surrounds the heart known as the pericardium. It is the rarest form of mesothelioma and only accounts for one percent of all diagnoses.
What is Pericardial Mesothelioma?
While pleural and peritoneal mesothelioma are the most common forms, there have been less than 150 confirmed cases of pericardial mesothelioma in the medical literature from the last 30 years. The decades of research on mesothelioma has shown that almost all instances of mesothelioma can be linked directly to the ingestion or inhalation of asbestos fibers.
Researchers are still investigating the link between asbestos exposure and the development of pericardial mesothelioma, including how exactly asbestos fibers get into the pericardium. According to a study conducted in 2017, men are almost two times more likely to be diagnosed with pericardial mesothelioma than women.
How Is Pericardial Mesothelioma Treated?
Pericardial mesothelioma is so rare and often only diagnosed once it has progressed to later stages, which makes surgical options extremely limited. A pericardiectomy is a process of removing the pericardium and any surrounding cancerous tissue. It is typically only performed if the tumors are small and localized to the pericardium.
Chemotherapy is the most common treatment option for pericardial mesothelioma patients. Chemotherapy is most effective when used in conjunction with another treatment like a pericardiectomy surgery.
Since pericardial mesothelioma is extremely difficult to operate on, most of the patients diagnosed with the disease are given palliative treatments to manage pain and make them as comfortable as possible. This includes procedures like a pericardiocentesis which is meant to alleviate pressure from fluid build-up around the heart.
People diagnosed with pericardial mesothelioma typically do not experience any symptoms until cancer develops past the initial stages. Once symptoms start to present themselves, the disease can be confused with other heart conditions.
The most common symptom is the buildup of fluid around the heart known as pericardial effusions, which can hinder normal cardiac function and can be extremely painful. Other common symptoms experienced by patients with pericardial mesothelioma include:
- Chest Pain
- Shortness of breath (dyspnea)
- Swelling of face or arms
- A cough
- Pericardial effusion
- Irregular heartbeat (arrhythmia)
If you are experiencing any of these symptoms, particularly those relating to your heart, seek medical attention as soon as possible. Doctors will perform CT Scans, X-rays, or an echocardiogram, which are all able to detect the symptoms’ root cause so that the problem can be accurately diagnosed.
How is Pericardial Mesothelioma Diagnosed?
Physicians have a difficult time diagnosing pericardial mesothelioma because of how rare the disease is and how symptoms do not typically show themselves until the malignancy has grown to stage III or IV.
Doctors use imaging tests like a CT scan, X-ray, and echocardiogram to determine if a patient has pericardial mesothelioma. Computed tomography (CT) scans are usually the most effective in detecting pericardial tumors.
Echocardiograms use sound waves to monitor heart ailments, including atrial fibrillation, heart disease, and pericardial mesothelioma.
An echocardiogram allows the specialist to hear how a patient’s heart is beating to ensure it is pumping enough blood.
Many patients with pericardial mesothelioma experience chest pain because their heart is unable to pump blood at maximum capacity due to pressure from pericardial effusions. Echocardiograms are essential in determining the extent to which the heart itself has been affected by mesothelioma.
Imaging tests can show certain abnormalities that fit the characteristics of pericardial mesothelioma, but fluid and tissue biopsies are the only way to confirm the results of initial scans.
Patients with pericardial mesothelioma commonly complain about chest pain. This pain is caused by the increased pressure on the heart and chest cavity from the buildup of fluid in the pericardial sac. When mesothelioma is suspected, cardiac surgeons will typically extract this fluid for cytology.
However, there is a chance that no malignant cells will be in that particular fluid sample so tissue biopsies are most often recommended if the first fluid biopsy comes back negative. Fluid biopsies alone have been proven to be effective only about 24% of the time.
Tissue biopsies are a bit more invasive than fluid biopsies but taking tissue directly from the region suspected to be cancerous is the most definitive method for an accurate diagnosis.
Treatment options for pericardial mesothelioma are limited due to the proximity of the tumors to the heart. More than half of the patients who have been diagnosed with pericardial mesothelioma are not ideal candidates for surgery because the tumors are not localized.
Unlike pleural or peritoneal mesothelioma, where the lung or abdominal lining can be removed or parts of the lung itself can be removed without much risk to the patient, the pericardium presents further risks and can be more difficult to remove.
Most of the time, treatment includes chemotherapy and other palliative treatments for pain management if surgery is ruled out when cancer has progressed past stage III. Radiation is rarely ever used to treat pericardial mesothelioma because of the risk of toxicity to the heart.
A pericardiectomy is an extremely delicate surgical procedure meant to remove some or all of the pericardium. If patients are deemed eligible and their cancer has not progressed into the organ itself, they may be able to have tumors surgically removed along with the pericardium.
The point of this procedure is to relieve the constrictive pressure caused by the non-elastic malignancy which has overtaken the flexible pericardium. It is a very rare procedure because most pericardial mesothelioma tumors have already metastasized by the time it has been detected.
If the tumor has not metastasized beyond the point of origin, then a procedure known as a tumor resection can be performed where the nodule is removed without having to remove the pericardium.
According to a medical literature review from 2017, 46% of patients diagnosed with pericardial mesothelioma underwent surgery.
Chemotherapy for pericardial mesothelioma is one of the more standard options for treating this kind of mesothelioma, though the overall benefits are limited. Currently, there are two chemotherapy drugs, pemetrexed and cisplatin, which have shown to be the most effective in slowing or halting tumor growth in the pericardium.
Another chemotherapy drug for patients with pericardial mesothelioma is called gemcitabine, which has shown mixed results in clinical trials. That same study from 2017 showed that 39% of patients involved underwent some form of chemotherapy, often in conjunction with surgery for a multimodal therapy plan.
Since treatments for pericardial mesothelioma are so limited, the use of palliative treatments to manage pain and keep the patient comfortable is essential to giving them the best possible prognosis.
Most of the pain comes from the build-up of tissue in the pericardium that restricts normal heart function and can lead to arrhythmias and chest pain. Sometimes a procedure called a pericardiocentesis is used to remove excess fluid in the pericardium, improving cardiac functions.
What is the Prognosis?
The prognosis for patients with pericardial mesothelioma is poorer than for pleural and peritoneal mesothelioma. On average, less than 40% of patients diagnosed with this form of mesothelioma live longer than 6 months after the initial onset of symptoms.
According to a study by Dr. Raphael Bueno of Brigham & Women’s Hospital, surgical resections have been performed at early stages and can be curative for localized tumors.
Not much information is available to researchers about how exactly asbestos fibers could become lodged in the pericardium and which treatments are most effective. However, some patients show a positive outlook.
A 54-year old woman who, after undergoing a tumor resection for a localized malignancy as well as multiple rounds of chemotherapy, survived for 4 years without a recurrence of the disease, but did have two other independent nodules that formed in other regions of the pericardium. Another male patient who underwent a pericardiectomy survived five years after his initial surgery date.
There is also research being conducted through Johns Hopkins Hospital that shows pericardiectomies are being performed with lower mortality rates as time goes on and improvements are made to the surgical process.
Taking part in clinical trials can also offer unique treatments and is one-way patients might be able to improve their prognosis.
Learn more about pericardial mesothelioma in our free Mesothelioma Guide.