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Written By: Devin Golden

Malignant Pleural Mesothelioma

Malignant pleural mesothelioma is a rare cancer that forms in the lining of the lung, known as the pleura. Asbestos exposure is the only known cause. Microscopic asbestos fibers can be inhaled and then become embedded or lodged in the pleura. Early diagnosis and treatment of mesothelioma provides for the best outcomes. Treatment of patients with malignant pleural mesothelioma include surgery, chemotherapy, and immunotherapy.

Dr. Abraham Lebenthal

Medically Reviewed By

Dr. Abraham Lebenthal

Mesothelioma Thoracic Surgeon

Dr. Abraham Lebenthal

Medically Reviewed By

Dr. Abraham Lebenthal

Mesothelioma Thoracic Surgeon

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Important Facts About Pleural Mesothelioma

  • Pleural mesothelioma is the most common type of mesothelioma, accounting for approximately 75-80% of all mesothelioma cases.
  • The only known cause of malignant mesothelioma is asbestos exposure.
  • Around 80% of patients diagnosed with pleural mesothelioma are males.
  • Treatments for pleural mesothelioma include surgery, chemotherapy, immunotherapy, radiation and tumor treating fields.
  • Median survival time is usually 1-2 years depending on stage of cancer, patient’s age, overall health status, and therapies used to treat pleural mesothelioma. Some patients survive much longer, and the 5-year survival is 10% and rising as treatment improves.
  • Aggressive treatments improve overall survival from pleural mesothelioma. Nearly 50% live for at least one year.

What Is Pleural Mesothelioma?

Pleural mesothelioma is a cancer of the pleura, which is the thin membrane between the chest wall and lungs. This specific cancer of the pleura accounts for nearly 80% of all cases of mesothelioma.

Malignant pleural mesothelioma is a rare cancer that can spread to other parts of the body. The average life expectancy of a patient is 11-20 months.

Patients with pleural mesothelioma have been able to live multiple years due to:

  • Evolving surgical techniques
  • Improvements in chemotherapy, immunotherapy and radiation
  • The rise of tumor treating fields

Malignant Pleural Mesothelioma Causes

The main known cause of pleural mesothelioma is exposure to asbestos. Asbestos products are usually found in homes and businesses built prior to the 1980s. There are multiple types of asbestos exposure, but occupational asbestos exposure is the most common cause of mesothelioma.

When asbestos fibers are inhaled, they often travel to the lungs. However, these fibers sometimes enter the pleural space, which includes mesothelial cells. These microscopic fibers cause irritation to cells and cell mutations occur.

While mesothelial cells often absorb unwanted particles, they struggle to expel asbestos fibers. The irritable asbestos particles can cause the mesothelial cells in the pleura to mutate and develop into cancer cells. These cancer cells clump together and form tumors.

Development of Pleural Mesothelioma

Inhaled asbestos fibers enter the body and travel to the lungs
Inhaled asbestos fibers enter the body and travel to the lungs
Asbestos fibers enter the pleural space, interacting with the cells
Asbestos fibers enter the pleural space, interacting with the cells
The asbestos fibers begin to irritate the cells, causing them to form tumors
The asbestos fibers begin to irritate the cells, causing them to form tumors
Tumors start to form and begin to spread along the pleural ling of the lung
Tumors start to form and begin to spread along the pleural ling of the lung

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Pleural Mesothelioma Symptoms

Signs and symptoms of pleural mesothelioma may include shortness of breath, coughing and chest pain. These symptoms can also be similar to other common illnesses, such as pneumonia.

Other pleural mesothelioma symptoms include:

  • Pain in the lower back
  • Fatigue
  • Unexplained weight loss
  • Swelling of the face or arms
  • Fluid buildup in the pleural space
  • Night sweats
  • Hoarseness
  • Trouble swallowing
Explaining Early Signs of Pleural Mesothelioma Icon

Explaining Early Signs of Pleural Mesothelioma

One of the toughest parts of fighting pleural mesothelioma is detecting this rare cancer. Early detection is important but also challenging. Not many know the symptoms of mesothelioma, and some disregard them as more common health issues.

There are a few early signs of pleural mesothelioma. If you react quickly and get diagnosed soon enough, you may be eligible for surgery and other aggressive treatment.

Pleural Effusions

Pleural effusions are buildup of fluid between the pleural layers. This area is where pleural mesothelioma forms. It’s a narrow lining of tissue and fluid. When tumors form, they take up the already-limited space and push fluid into pockets.

Doctors can notice pleural effusions on imaging scans, such as an X-ray or computed tomography (CT) scan.

pie chart with the number 60% in the middle

Pleural effusions can be one of the first diagnostic signs of pleural mesothelioma. A report in ScienceDirect found 60% of diagnosed pleural mesothelioma cases had this symptom.

Pleural Thickening

Pleural thickening is typically the result of chronic inflammation, infection or injury in the pleura. Injury to the pleura can come from previous infections and a history of asbestos exposure. Exposure to asbestos can cause inhalation of small asbestos fibers that get into the respiratory system, penetrate the mesothelial cell lining.

pie chart with the number 80% in the middle

Pleural mesothelioma forms from irritation and inflammation of pleural tissues. The thickened pleural tissue can be detected through a chest X-ray or CT scan. The ScienceDirect report found pleural thickening in 88% of diagnosed cases.

Pleural Mesothelioma Diagnosis

The first step in diagnosing pleural mesothelioma is imaging tests, such as an X-ray or CT scan. If the test results show abnormalities or possible cancerous tumors, doctors will recommend a tissue biopsy.

A biopsy involves taking small pieces of tissue to test for cancer cells. A biopsy also helps doctors determine details about the cell histology and possibly the stage of the disease (if the biopsy is of a lymph node).

Doctors may also extract a fluid sample to look for cancerous cells. However, this method is less reliable than a tissue biopsy.

Pleural Mesothelioma Patients Icon

Pleural Mesothelioma Patients

According to the American Society of Clinical Oncology, the median age of patients with pleural mesothelioma is 72. This is at the time of their diagnosis.

The disease’s latency period — is time it takes to develop mesothelioma after a patient is exposed to asbestos — is 20-50 years. This lengthy development time is why many patients are older in age.

illustration of 10 people, 8 men and 2 women

According to another study, around 80% of patients are male. This is largely due to occupational exposure to asbestos.

Can Pleural Mesothelioma Spread to Other Organs? Icon

Can Pleural Mesothelioma Spread to Other Organs?

Malignant pleural mesothelioma can spread to the lymph nodes, which can cause cancer to spread throughout the body. The disease also spreads to the nearby lung, heart and diaphragm.

The 4 Stages of Pleural Mesothelioma Icon

The 4 Stages of Pleural Mesothelioma

Stage 1

This is an early stage for pleural mesothelioma. Tumors are only found in the pleural cavity, which is the disease’s point of origin. The cancer likely just developed and has not had enough time to spread to other tissue. Detection at this stage is difficult.

Stage 2

This is still an early stage cancer and is mostly limited to the point of origin, but some localized lymph node involvement begins. Tumors may begin spreading into the lung cavity.

Stage 3

The disease is no longer localized, metastasizing to the nearby lung, the diaphragm and lymph nodes is likely.

Stage 4

This cancer stage can have disease that has spread to nearby structures like lungs, heart, major blood vessels, or spine. The cancer has also spread to at least one distant area. Treatment may be limited at this stage.

Pleural Mesothelioma Treatment Options

There are several pleural mesothelioma treatment options, including surgical operations, chemotherapy, immunotherapy, radiation and tumor treating fields.

Surgery Icon

Surgery

There are two primary surgical options for malignant pleural mesothelioma patients: extrapleural pneumonectomy and pleurectomy with decortication. The former removes the affected lung while the latter preserves this organ.

Chemotherapy Icon

Chemotherapy

The chemotherapy drugs approved by the U.S. Food and Drug Administration (FDA) for malignant pleural mesothelioma are pemetrexed and cisplatin. Chemotherapy is often used in combination with other treatment options.

Immunotherapy Icon

Immunotherapy

The FDA approved Opdivo and Yervoy for pleural mesothelioma. These two drugs are immunotherapies. They are available to patients who cannot have surgery for their cancer. Other types of immunotherapy are in clinical testing.

Radiation Icon

Radiation

Scientists are developing radiation techniques to target tumors and leave healthy tissue unharmed. Radiation is also used for palliative purposes.

Tumor Treating Fields Icon

Tumor Treating Fields

Tumor treating fields send electromagnetic waves through the body to disrupt cancer cell growth. The FDA approved a tumor treating fields device, called Optune Lua, in 2019 for malignant mesothelioma. The therapy is for patients who cannot have surgery.

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A nation wide list of sites where you or a loved one may have come in contact with asbestos.

Surgery for Pleural Mesothelioma

Surgical management of malignant pleural mesothelioma is the best option for patients to improve their prognosis. The two surgical options for pleural mesothelioma are extrapleural pneumonectomy (EPP) and pleurectomy with decortication (P/D). The EPP removes the entire affected lung while the P/D surgery preserves this organ.

Not all patients are surgical candidates, these patients may still receive procedures to provide symptoms relief. A thoracentesis or pleurodesis are surgical procedures used to drain fluid from the pleura and improve comfort.

Extrapleural Pneumonectomy (EPP)

Extrapleural pneumonectomy is an aggressive surgical treatment for malignant pleural mesothelioma. During EPP, surgeons remove the entire diseased lung. During the surgery, the physician will also remove the pleura, part or all of the diaphragm, and the pericardium.

The goal of EPP is to remove as much, if not all, of the cancer. There are typically many small mesothelioma tumors spread out around the lung, which is why removing all the tumors is so challenging for surgeons.

Patients often receive chemotherapy as part of a multimodal treatment approach for their pleural mesothelioma. Chemotherapy provided prior to surgery is neoadjuvant cancer treatment. This is given in an attempt to slow or stop the progression of the disease and shrink tumors prior to surgery.

If chemotherapy is used as a post-surgical therapy, it is called adjuvant treatment. Chemotherapy after surgery is given in an attempt to kill any remaining cancer cells not removed during the surgical resection.

Dr. David Sugarbaker perfected EPP. Until his death in 2018, he was one of the leading mesothelioma specialists in the country.

Pleurectomy With Decortication (P/D)

Pleurectomy with decortication is an alternative to EPP. It was developed for malignant pleural mesothelioma when EPP is deemed too risky. Many doctors now prefer the P/D method over EPP since it promotes a better quality of life.

P/D is most effective for early-stage pleural mesothelioma. The surgery spares their lungs and only takes out the pleura, where most of the cancer is located.

Some patients may need their pericardium and part of their diaphragm taken out during P/D. This surgery is called an “extended pleurectomy with decortication.” It’s effective when the cancer has spread beyond the pleural space.

Patients may receive chemotherapy as a pre or post-surgical therapy.

Chemotherapy for Pleural Mesothelioma

Chemotherapy was the first treatment option approved by the U.S. Food and Drug Administration (FDA) for malignant pleural mesothelioma. The chemotherapy drugs approved are pemetrexed and cisplatin. These drugs are approved for intravenous use.

chemotherapy icon depicting chemotherapy drug in an IV drip bags.

Doctors may substitute cisplatin with carboplatin if needed. Chemotherapy is often used in combination with other treatments.

Another chemotherapy option is called hyperthermic intrathoracic chemotherapy (HIPEC). This involves a heated chemotherapy solution that is delivered directly into the chest cavity.

Immunotherapy for Pleural Mesothelioma

Immunotherapy is the newest FDA-approved treatment for malignant pleural mesothelioma. The FDA approved Opdivo (nivolumab) and Yervoy (ipilimumab) for unresectable cases. Unresectable means the patients do not qualify for surgery.

immunotherapy drug icon depicting medication in a syringe

Opdivo and Yervoy are immune checkpoint inhibitor drugs, a type of immunotherapy drug that can target specific types of cancer, for malignant pleural mesothelioma. Immune checkpoint inhibitors help fight against the proteins on the pleural mesothelioma cancer cells that inhibit the normal attack from the immune system.

Opdivo and Yervoy block the cancer surface proteins PD-L1 and B7 from latching onto PD-1 and CTLA-4. Another immunotherapy drug, Keytruda (pembrolizumab), is approved for cases with a high tumor burden volume. This immune checkpoint inhibitor also blocks PD-L1.

Opdivo and Yervoy have impressive results, improving the outcomes for pleural mesothelioma patients. The combination led to an average survival of 18-19 months in large-scale mesothelioma studies. This outperforms chemotherapy by 4-5 months.

Radiation for Pleural Mesothelioma

Radiation for malignant pleural mesothelioma is one of the oldest treatment options offered to patients. It’s best used either before or after another therapy, such as surgery, chemotherapy or immunotherapy. Radiation can shrink the size of tumors before surgery or reduce the chance of recurrence after surgery, it can also be used to relieve symptoms as a palliative treatment.

Icon depicting radiation treatment

Most mesothelioma doctors don’t use radiation on its own to improve survival. Radiation is also used for palliative purposes to ease pain caused by fluid buildup or tumor pressure. This can shrink tumors and alleviate pressure in the chest.

Scientists are developing radiation techniques to target tumors and leave healthy tissue unharmed. Types of radiation therapy such as intensity-modulated radiation or proton radiation reduce side effects and long-term health problems.

Doctors may also use intraoperative radiotherapy for malignant pleural mesothelioma. It’s best used with P/D surgery. Doctors send radiation directly through the incision and into the chest cavity.

Tumor Treating Fields for Pleural Mesothelioma

The FDA approved a tumor treating fields device called Optune Lua in 2019 under a Humanitarian Device Exemption ruling. It was originally called NovoTTF-100L System.

icon depicting an electric field

The device is for non-surgical cases of malignant pleural mesothelioma. It must be used in tandem with intravenous chemotherapy drugs pemetrexed and cisplatin (or carboplatin if needed).

The device sends electric fields into the patient’s chest region where the cancer is located. These waves disrupt the growth and division of cancer cells in the pleura and around the lungs to kill cancer cells.

The Optune Lua device is non-invasive and is worn for 23 hours a day. It is only approved when surgery isn’t an option for treatment, and it must be used with chemotherapy. Nearly 100% of patients in a study had a positive response to the therapy. The survival statistics outperform chemotherapy on its own.

Pleural Mesothelioma Prognosis

A pleural mesothelioma prognosis is generally poor. The doctor will identify the type of mesothelioma and your treatment options, with this information doctors will then determine an estimate of the patient’s life expectancy. It’s partly based on the experience of past patients.

Many factors play a role in a patient’s pleural mesothelioma prognosis:

  • Cell type
  • Stage of cancer
  • Age
  • Overall health
  • Treatments
Pleural Mesothelioma Survival Rates Icon

Pleural Mesothelioma Survival Rates

Since surgery directly removes tumors, it is considered the most effective at extending life and improving pleural mesothelioma survival rates. However, some patients cannot undergo surgery due to numerous factors, as surgery could lead to additional health concerns.

pie chart with the number 40% inside

Approximately 40% of malignant pleural mesothelioma patients live for at least one year following their diagnosis. Patients who undergo treatment for malignant pleural mesothelioma can outlive their prognosis.

One study published in the Annals of Thoracic Surgery reported half of patients living for three years after P/D.

Fortunately, more patients have access to and are receiving treatment for pleural mesothelioma. In 2004, only 34% of patients received treatment for their cancer. In 2014, the number rose to 54%.

The one-year survival of patients with malignant pleural mesothelioma rose from 37% to 47%, and three-year survival rates rose from 9% to 15%.

Surgery is not an option for many people with malignant pleural mesothelioma. Chemotherapy, immunotherapy and tumor treating fields are often the best options for those patients. Most patients respond best to immunotherapy, which has an average survival of around 18 months.

Tumor treating fields led to a similar survival average: 18 months. People with the epithelioid mesothelioma cell type performed the best, with survival of around 21 months.

These therapies outperform chemotherapy for non-surgery cases, which are usually stage 3 and stage 4. Traditionally, these cases had an average survival time of 12-14 months, so improved survival thanks to immunotherapy and tumor treating fields is a promising direction for pleural mesothelioma prognosis.

The most effective way to improve your prognosis is to start treatment immediately. If you have been diagnosed with this form of mesothelioma, then you should find a mesothelioma specialist for a consultation or second opinion. You may learn your disease is at an earlier stage than you originally were told, and you could have more treatment options available.

Veterans With Pleural Mesothelioma

The largest group (around 33%) of people diagnosed with mesothelioma is military veterans. Asbestos was an insulant in military vehicles, naval vessels and army bunkers. The mineral was so widely used in the military that avoiding airborne asbestos fibers was close to impossible.

militray icon

Due to the high prevalence of veteran mesothelioma patients, the U.S. Department of Veterans Affairs (VA) provides financial benefits to victims. Veterans who have this cancer can:

  • Receive disability benefits, if their disease is service-related
  • Find a specialist within the VA health care system
  • Receive pension if their mesothelioma is not service-related
  • Set up their surviving loved ones to receive benefits if they die from mesothelioma

Learn more about what veterans can do to support their families and live longer in our free Veteran’s Support Guide.

Frequently Asked Questions About Pleural Mesothelioma

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How can you tell if you have malignant pleural mesothelioma?

Identifying the common symptoms of pleural mesothelioma is the first way to detect this cancer. Typical signs and symptoms of mesothelioma of the pleura include:

  • Persistent cough
  • Chest pain
  • Night sweats
  • Difficulty breathing
  • Fluid buildup around the lungs
  • Weight loss
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How long do people live with pleural mesothelioma?

The average prognosis for pleural mesothelioma is a little more than one year, although it varies based on stage of the cancer and health of the patient. Treatments also can extend survival and life expectancy for multiple years.

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How is pleural mesothelioma diagnosed?

Pleural mesothelioma develops in the thin lining of the thoracic cavity. Physicians perform different imaging scans to detect the presence of cancer. If the scans detect abnormalities then the next step is a tissue biopsy. A tissue biopsy is the only way to get a definitive diagnosis of mesothelioma.

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What causes pleural mesothelioma?

Asbestos is the only known cause of pleural mesothelioma. Sharp, microscopic asbestos fibers lining of the lungs and irritate cells. Pleural mesothelioma forms in the pleura, which is a narrow membrane between the lung cavity and chest wall. If asbestos fibers enter the pleura, the body has difficulty expelling them.

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Who is most likely to get pleural mesothelioma?

The demographic most affected by pleural mesothelioma is elderly men. The cancer can take up to 50 years to form after asbestos exposure occurs, meaning most people are diagnosed in their 60s and 70s. Men are also most at risk due to working jobs that exposed them to asbestos regularly.

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How is pleural mesothelioma treated?

People with pleural mesothelioma often receive a combination of surgery, chemotherapy and radiation. This is called multimodal treatment, which is best for extending survival. Immunotherapy and tumor treating fields are other treatments for pleural mesothelioma. The FDA approved three immunotherapy drugs and one tumor treating fields device for many malignant pleural mesothelioma cases.

Sources & Author

  1. Pleural Mesothelioma. ScienceDirect. Retrieved from: https://www.sciencedirect.com/topics/medicine-and-dentistry/pleura-mesothelioma. Accessed: 07/15/2021.
  2. Diagnostic Approach to Pleural Effusions in Adults. American Family Physician. Retrieved from: https://www.aafp.org/afp/2006/0401/p1211.html. Accessed: 07/15/2021.
  3. Life Expectancy in Pleural and Peritoneal Mesothelioma. Lung Cancer International. Retrieved from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5292397/. Accessed: 03/19/19.
  4. Presentation, initial evaluation, and prognosis of malignant pleural mesothelioma. UpToDate. Retrieved from: https://www.uptodate.com/contents/presentation-initial-evaluation-and-prognosis-of-malignant-pleural-mesothelioma?topicRef=4625&source=see_link. Accessed: 03/19/19.
  5. Tests for Malignant Mesothelioma. American Cancer Society. Retrieved from: https://www.cancer.org/cancer/malignant-mesothelioma/detection-diagnosis-staging/how-diagnosed.html. Accessed: 04/10/19.
  6. Signs and Symptoms of Mesothelioma. American Cancer Society. Retrieved from: https://www.cancer.net/cancer-types/mesothelioma/statistics. Accessed: 03/19/19.
  7. Mesothelioma: Statistics. American Society of Clinical Oncology. Retrieved from: https://www.cancer.org/cancer/malignant-mesothelioma/detection-diagnosis-staging/how-diagnosed.html. Accessed: 04/10/19.
  8. Veterans Asbestos Exposure. U.S. Department of Veterans Affairs. Retrieved from: https://www.va.gov/disability/eligibility/hazardous-materials-exposure/asbestos/. Accessed: 04/20/2023.
  9. Debunking The Myths About Mesothelioma Procedure Pleurectomy and Decortication. Pacific Heart, Lung and Blood Institute. Retrieved from: https://www.cancer.org/cancer/malignant-mesothelioma/detection-diagnosis-staging/how-diagnosed.html. Accessed: 05/02/19.
  10. Extended pleurectomy decortication: the current role. Translational Lung Cancer Research. Retrieved from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6204414/. Accessed: 08/27/19.
  11. Extended Pleurectomy-Decortication–Based Treatment for Advanced Stage Epithelial Mesothelioma Yielding a Median Survival of Nearly Three Years. The Annals of Thoracic Surgery. Retrieved from: https://www.annalsthoracicsurgery.org/article/S0003-4975(16)31139-0/fulltext. Accessed: 08/26/19.
  12. Extrapleural Pneumonectomy. University of California San Francisco Department of Surgery. Retrieved from: https://surgery.ucsf.edu/procedure/extrapleural-pneumonectomy. Accessed: 12/12/2023.
Devin Golden

About the Writer, Devin Golden

Devin Golden is a content writer for Mesothelioma Guide. He produces mesothelioma-related content on various mediums, including the Mesothelioma Guide website and social media channels. Devin's objective is to translate complex information regarding mesothelioma into informative, easily absorbable content to help patients and their loved ones.