Pericardial mesothelioma is an extremely rare form of asbestos cancer that develops in the pericardiaum, a membrane that lines the heart. It is one of the rarest forms of mesothelioma, accounting for 1 – 2% of all diagnoses. Prognosis is poor, with most cases being diagnosed only after an autopsy.
Primary pericardial mesothelioma is extremely rare, with fewer than 50 people diagnosed each year. While primary pericardial mesothelioma is rare, pericardial involvement by mesothelioma most often results from extension and spread of pleural mesothelioma. On average, patients survive about six months after diagnosis. With so few cases, research is limited and conventional treatments, like surgery and chemotherapy, are often the standard course of treatment.
What is the Prognosis for Pericardial Mesothelioma?
Though all mesothelioma patients face a poor prognosis, primary pericardial mesothelioma has the worst life expectancy. The median survival period for peritoneal mesothelioma is six months, and there are only a few case reports of patients who survived one year or longer.
The most important factors affecting the prognosis of pericardial mesothelioma patients are stage of disease, cell type and patient characteristics, like age and gender.
|1 year after diagnosis||51%|
|3 years after diagnosis||26%|
|5 years after diagnosis||23%|
Pericardial Mesothelioma Symptoms
As with all forms of malignant mesothelioma, it may take years for pericardial mesothelioma symptoms to present. Due to its rarity, researchers are unsure of how asbestos fibers reach the pericardium in cases of primary pericardial mesothelioma. The symptoms patients experience may be more varied for instances of secondary pericardial tumors.
Pericardial mesothelioma develops in between two layers of delicate membrane around the heart. Studies have found the tumors initially thicken the membrane, which may limit heart function and lead to more severe symptoms, like cardiac failure. Because the disease develops in the heart lining, patients may experience severe symptoms even at earlier stages.
- Difficulty breathing (dyspnea)
- Chest pain
- Night sweats
- Decreased appetite and weight loss
- Pericardial effusion
- Cardiac tamponade (compression of the heart by fluid)
- Constrictive pericarditis (inflammation of the pericardium)
How is Pericardial Mesothelioma Diagnosed?
Due to its rarity, all forms of mesothelioma cancer are difficult to diagnose, though malignant pericardial mesothelioma presents more challenges. Because the cancer impacts the heart, worsening symptoms can have a severe impact on the body quickly as the disease progresses. Unfortunately, many pericardial mesothelioma patients aren’t diagnosed until an autopsy is performed, with one report estimating about 10 – 20% of cases are properly diagnosed before a patient’s death.
Similarly to peritoneal and pleural mesothelioma, diagnosis typically begins with imaging tests, like x-rays and CT scans. These scans may help identify pericardial effusion, or excess fluid around the heart, or any visible tumors. Blood tests may also be performed to help differentiate mesothelioma from other conditions.
A biopsy is the most important step for an accurate mesothelioma diagnosis. A doctor takes a tissue sample for analysis under a microscope. As part of the process, a pathologist will determine cell type and how the disease can be expected to advance.
Pericardial Mesothelioma Stages
Determining the stage of pericardial mesothelioma is an important aspect of the diagnostic process, as it helps establish prognosis and treatment options. Since there have been so few reported cases, pericardial mesothelioma does not have a formal staging system, but doctors may use more general cancer and mesothelioma characteristics to determine how advanced the cancer is.
Stage 1 or stage 2 pericardial mesothelioma will indicate more localized disease. With limited metastasis, if any, patients will have more treatment options, like surgical resection. Because the disease is difficult to diagnose, many documented cases of pericardial mesothelioma indicate a late stage diagnosis, where the cancer has spread to nearby organs and the lymph nodes. As the disease becomes more advanced, patients may only have palliative treatment options available.
What Treatments are Available for Pericardial Mesothelioma?
Like the more common forms of the cancer, pericardial mesothelioma is often treated with standard treatments like surgery and chemotherapy. Because most patients are diagnosed at a more advanced stage, these treatments are often applied palliatively to prolong life, reduce symptoms and improve quality of life.
For pericardial mesothelioma patients diagnosed at an earlier stage, surgeries like a pericardiectomy may be viable as part of a combined modality treatment plan. During a pericardiectomy, a mesothelioma specialist will remove all or part of the lining of the heart. Ideally, the process would remove all visible tumors.
Pericardiectomy is commonly followed by combination chemotherapy to kill remaining cancer cells. A 2017 review of 103 pericardial mesothelioma cases found that 46% of patients underwent surgery, and 37% were treated with chemotherapy. Both treatments showed improved survival from the average six months, with one case report noting a patient who survived at least 1.5 years and another recent report discussing a patient who has survived four years so far after surgical resection.
Palliative surgeries, such as a pericardiocentesis, may also be an option for some patients. Pericardial effusion, fluid buildup around the heart, is a common symptom of the disease, which can put pressure on the heart and negatively impact heart function. A pericardiocentesis can relieve pressure on the heart by draining the excess fluid. The procedure is minimally invasive, as a needle is inserted into the pericardium and the fluid is then drained through a catheter.
Radiation therapy is also occasionally applied, but has not shown success in extending life expectancy. For some patients, it may be a viable option to reduce symptoms.
Clinical trials specifically for pericardial mesothelioma are not feasible since there are so few cases diagnosed each year. In many instances, pericardial mesothelioma patients are able to participate in clinical trials for pleural mesothelioma. Also, it is expected that newer therapies being used in other mesothelioma types, such as immunotherapy, will have similar effects in pericardial mesothelioma and represent treatment options.
Author: Linda Molinari
Editor in Chief, Mesothelioma Cancer AllianceRead about Linda
Reviewer: Dr. James Stevenson
Medical Reviewer and Thoracic Medical OncologistRead about Dr. Stevenson
Ashinuma H, Shingyoji M, et al. Endobronchial Ultrasound-guided Transbronchial Needle Aspiration in a Patient with Pericardial Mesothelioma. Internal Medicine. 2015; 54(1):43-8. doi: 10.2169/internalmedicine.54.3216
Freitas DMM, Ramos RL, et al. Primary pericardial mesothelioma and asbestos exposure: a rare fatal disease. BMJ Case Report. May 2017. doi: 10.1136/bcr-2017-219949
Gong W, Ye X, et al. Primary malignant pericardial mesothelioma—a rare cause of superior vena cava thrombosis and constrictive pericarditis. Journal of Thoracic Disease. December 2014; 6(12): E272–E275. doi: 10.3978/j.issn.2072-1439.2014.11.36
Karpathiou G, Mobarki M, et al. Pericardial and Pleural Metastases: Clinical, Histological and Molecular Differences. The Annals of Thoracic Surgery. May 2018. pii: S0003-4975(18)30719-7. doi: 10.1016/j.athoracsur.2018.04.073
Kim JS, Lim SY, et al. A Case Report of Primary Pericardial Malignant Mesothelioma Treated with Pemetrexed and Cisplatin. Journal of Korean Medical Science. November 2017; 32(11):1879-1884. doi: 10.3346/jkms.2017.32.11.1879
Li X, Lu R, et al. 18F-fluorodeoxyglucose imaging of primary malignant pericardial mesothelioma with concurrent pericardial and pleural effusions and bone metastasis: A case report. Molecular and Clinical Oncology. June 2018; 8(6):725-728. doi: 10.3892/mco.2018.1604
McGehee E, Reisch J, et al. Treatment and outcomes of primary pericardial mesothelioma: A systematic review. Journal of Clinical Oncology. 2017 35:15_suppl, e20027-e20027
Neragi-Miandoab S, Kim J, et al. Malignant Tumours of the Heart: A Review of Tumour Type, Diagnosis and Therapy. Clinical Oncology. December 2007; 19(10): 748-756. doi: 10.1016/j.clon.2007.06.009
Ramachandran R, Radhan P, et al. A rare case of primary malignant pericardial mesothelioma. Journal of Clinical Imaging Science. August 2014;4:47. doi: 10.4103/2156-7514.139737
Rodríguez M, Mallidi HR, et al. Recurrence of Pericardial Mesothelioma Affecting the Myocardium after Pericardial Resection. Annals of Thoracic Surgery. May 2018. doi: 10.1016/j.athoracsur.2018.04.057
Suman S, Schofield P, et al. Primary pericardial mesothelioma presenting as pericardial constriction: a case report. Heart. January 2004; 4; 90(1).