Dr. James Stevenson, M.D.
Parts of Lung Affected by Pleural Mesothelioma
What Is Pleural Mesothelioma?
Pleural mesothelioma is a type of cancer caused by asbestos fibers becoming embedded in the lining of the lungs. Over time, the fibers may cause inflammation and scarring. As the scarring worsens, it may develop into mesothelioma tumors.
- Pleural malignant mesothelioma is the most common form of mesothelioma cancer.
- Each year, about 2,500 people are diagnosed with the disease.
- Symptoms of the cancer commonly include shortness of breath, chest pain, dry cough and fatigue.
- Diagnosis typically consists of multiple tests, including scans and biopsies.
- Pleural mesothelioma is often treated with chemotherapy, surgery, radiation therapy and immunotherapy.
- A patient’s prognosis will vary depending on their individual case, with an average life expectancy of six to twelve months.
What Is the Prognosis for Pleural Mesothelioma?
As with all types of malignant mesothelioma, prognosis for pleural malignant mesothelioma is poor. For patients who do not receive treatment, the median survival time is six months. However, certain types of treatment can improve life expectancy, such as surgery combined with chemotherapy.
|Pleural Mesothelioma Survival Rates|
|1 year after diagnosis||73%|
|3 years after diagnosis||23%|
|5 years after diagnosis||12%|
|10 years after diagnosis||4.7%|
The most important factors affecting the prognosis of pleural malignant mesothelioma patients are:
Most pleural mesothelioma patients are diagnosed with the epithelioid cell type, which is the most common. Epithelioid cells typically form in solid sheets or cord arrangements, meaning they adhere closely together and don’t metastasize as quickly. They are also the most responsive to treatment. Typically, pleural mesothelioma patients with the epithelioid cell type survive 19 months.
The other cell types, sarcomatoid and biphasic, are less common and indicate a worse prognosis than epithelioid. Sarcomatoid mesothelioma doesn’t respond well to treatment and metastasizes aggressively, leaving patients with an average prognosis of 8 – 10 months.
Patients with biphasic pleural mesothelioma experience an intermediate life expectancy, depending on whether epithelioid or sarcomatoid cells are more dominant.
According to recent data, within the last decade, malignant pleural mesothelioma patients have been surviving longer overall as available treatments and diagnostic methods improve. Some patients are now becoming long-term survivors. Heather Von St. James was diagnosed with pleural mesothelioma in 2005 with an initial prognosis of 15 months. Following her treatment, she is now a survivor of more than a decade.
What Are the Symptoms of Pleural Mesothelioma?
After asbestos exposure, it can take 10 to 50 years for pleural mesothelioma symptoms to present. Symptoms typically first present in the chest cavity and respiratory system.
As the disease becomes more advanced, new and worsening symptoms may arise. For instance, stage 4 pleural mesothelioma symptoms may include coughing up blood and difficulty swallowing.
Pleural malignant mesothelioma patients may be diagnosed with a co-occurring asbestos-related condition, which can impact symptom onset. These include:
- Pleural plaques – Chalky substance that forms on the pleura due to a buildup of minerals, known as calcification
- Diffuse pleural thickening (DPT) – Gray, fibrous tissue that fills in pleural spaces
- Asbestosis – Scarring of the lungs (fibrosis)
These conditions may also develop independently of pleural mesothelioma.
Resources for Pleural Mesothelioma Patients
How Is Pleural Mesothelioma Diagnosed?
Diagnosing pleural mesothelioma often consists of multiple tests. One or more imaging scans, such as an X-ray or CT scan, may be performed first to identify tumors or metastasis (spreading of disease). If a tumor is detected, blood tests may be performed to look for certain biomarkers (high levels of specific substances in the blood), which can help differentiate mesothelioma from other conditions.
Currently, a biopsy is the only way to verify a malignant pleural mesothelioma diagnosis. Tests like a thoracentesis or thoracoscopy may be performed to take a tissue or fluid sample for analysis. For a thoracentesis, a doctor will insert a fine needle to remove fluid buildup in the chest.
A thoracoscopy is more invasive. Doctors insert a viewing tube called a thoracoscope into the chest to inspect the lungs and surrounding pleura. They can then remove a tissue sample or draw fluid for analysis. After the biopsy, a pathologist will study the cells to make a definitive diagnosis, including cell type and how the disease is expected to progress.
Pleural Mesothelioma Stages
As part of the diagnostic process, a mesothelioma specialist will also determine the stage of the disease, or how far it has spread. A patient’s stage is an important indicator of prognosis, and also helps determine available treatment options.
|Pleural Mesothelioma Life Expectancy by Stage|
|Stage 1||22.2 months|
|Stage 2||20 months|
|Stage 3||17.9 months|
|Stage 4||14.9 months|
The most common system used to determine pleural mesothelioma stage is the Tumor, Node, Metastasis or TNM staging system. Doctors will use the system to score a specific area of the body based on the size of the tumor, if the cancer has spread to the lymph nodes and if the cancer has spread to distant organs.
Stage 1 and stage 2 pleural mesothelioma indicate little to no metastasis, while advanced stages may have distant spreading to lymph nodes and other organs.
After the stage is determined, the medical team can develop an effective treatment plan for an individual’s case.
Pleural Mesothelioma Doctors
Finding a pleural mesothelioma doctor is an essential aspect of a patient’s care. Since the cancer is rare, many primary care physicians and oncologists have little to no experience with the disease. A specialist can accurately diagnose mesothelioma and develop personalized treatment plans, often using new research and clinical trials.
Top Pleural Mesothelioma Doctors in the Country
Chief, Division of Thoracic Surgery & Director, International Mesothelioma Program
Brigham and Women’s Hospital
Director, Department of Mesothelioma Program
The University of Texas MD Anderson Cancer Center
Pleural Mesothelioma Treatment
Pleural mesothelioma is typically treated with a multimodal approach, combining standard treatments like surgery, chemotherapy and radiation. A pleural mesothelioma treatment plan will largely depend on the cell type and stage of disease. Generally, treatment plans are not intended to cure the disease.
Mesothelioma surgery is common for pleural mesothelioma patients. The surgery may be aggressive with the goal of extending life expectancy, or less aggressive with the goal of palliating symptoms (relieving discomfort).
Surgery may be an option for early-stage malignant pleural mesothelioma patients. Pleurectomy/decortication (P/D) is one common surgical option that involves removal of the lining of the lung and chest wall, as well as other impacted tissues and organs.
Another option is an extrapleural pneumonectomy (EPP). The more aggressive procedure includes removing the affected lung, part of the diaphragm and the linings of the heart and lungs. Recent clinical trials have found these surgeries can extend life expectancy to three years or longer, especially when applied multimodally with chemotherapy and/or radiation.
Extrapleural Pneumonectomy (EPP) Success Story
Doctors often recommend chemotherapy as an additional treatment before or after surgery, or as a first-line therapy for patients with more advanced disease. A combination therapy of pemetrexed (Alimta®) and cisplatin is most commonly prescribed, though researchers continue to test new combinations. Radiation therapy may also be recommended for select patients to shrink tumors, which may also help relieve symptoms.
When determining treatment for pleural mesothelioma, patients should consider the cost. Reports show one course of treatment with Alimta® can cost upwards of $50,000, while surgeries like a pneumonectomy can cost at least $17,000.
Understanding treatment costs, along with the risks and benefits of available therapies, is crucial as patients and their loved ones make treatment decisions.
Emerging and Experimental Treatments
Because malignant pleural mesothelioma comprises the majority of mesothelioma cases, most experimental treatment options focus on this specific type. Promising treatments like immunotherapy, gene therapy and photodynamic therapy have shown early success in extending life expectancy in various clinical trials.
Studies are also advancing the efficacy of standard treatments. An early-phase clinical trial tested pleurectomy/decortication followed by a heated chemotherapy wash in patients with stage 1 and stage 2 pleural mesothelioma.
- A median survival of 20.3 months
- A median progression-free survival of 10.7 months
Though overall survival was about equal to the typical life expectancy for early-stage mesothelioma, the study noted extended survival time without disease progression when compared to standard treatments.
Studies continue to investigate mesothelioma treatment options to decrease recurrence rates and limit disease progression for patients.