Written By: Karen Selby, RN,
Last modified: July 21, 2021

What Is Pleural Mesothelioma?

Malignant pleural mesothelioma (MPM) is a cancer that develops in the chest cavity around the lungs. Almost 75% of diagnosed mesothelioma cases form in the pleura, making it the most common of the four types. Doctors diagnose roughly 3,000 new patients with MPM every year in the United States.

A thin tissue membrane, known as the pleura, lines the lungs and chest cavity. When asbestos fibers get lodged in the lining of the lungs, they can cause inflammation and scarring. The tumors that form in the pleura are known as pleural mesothelioma.

Where pleural mesothelioma forms
Where pleural mesothelioma forms

Pleural Mesothelioma Facts

  • Rarity: Pleural is the most common type of mesothelioma
  • Symptoms: Shortness of breath, chest pain, dry cough and fatigue
  • Life Expectancy: About 40% live at least one year

The cause of pleural mesothelioma is exposure to asbestos fibers, which a person can inhale into the lungs. It usually takes from 20 to 50 years for malignant pleural mesothelioma to develop after a person’s first exposure to asbestos. Because of this latency period, the disease usually affects people older than 75.

Average life expectancy for pleural mesothelioma is often less than 18 months, but it depends on many factors.

Although there is no cure for MPM, some patients live much longer with treatment. Combining several treatments, such as surgery, chemotherapy and radiation therapy, has helped some people live for years. Clinical trials offer access to new treatments such as immunotherapy.

What Causes Pleural Mesothelioma?

Asbestos exposure is the primary cause of malignant pleural mesothelioma. After the microscopic mineral fibers are inhaled, their sharp, pointed shape causes them to lodge in the lungs and gradually migrate into the pleural lining.

Over many years, these fibers cause irritation, chronic inflammation and genetic changes in DNA that turn healthy pleural cells cancerous.

The pleural lining of the lungs consists of two layers:

  • The outer layer lines the entire chest cavity under the ribs
  • The inner layer covers and protects the surface of the lungs

A malignant tumor can develop on either layer and may spread to the other layer. As nodules develop on the pleural surface, they grow to form tumor masses around the affected lung. The tumors can also cause pleural fluid to accumulate in the chest cavity.

Pleural mesothelioma developing in the lungs caused by inhaled asbestos fibers
Pleural mesothelioma tumors form when asbestos fibers lodge on the lining of the lungs.

The combination of tumor mass on the lung and a collection of pleural fluid increases pressure in the chest and prevents the lung from expanding, which causes breathing difficulties.

When left untreated, malignant cells can multiply and damage nearby healthy tissues or spread through the blood and lymph system, forming new tumors on distant organs.

Symptoms of Pleural Mesothelioma

For many people, mesothelioma symptoms are not noticeable until the cancer is in a later stage. 

Symptoms can include:

  • Shortness of breath
  • Chest pain or painful breathing
  • Persistent dry or raspy cough
  • Coughing up blood
  • Difficulty swallowing
  • Pain in the lower back or rib area
  • Unexplained weight loss and fatigue
  • Swelling of the face or arms
  • Night sweats or fever
  • Lumps under the skin on the chest

Patients rarely mention weight loss and fatigue during their initial doctor visit. These symptoms may only appear if the cancer is advanced. Some patients also develop swelling of the face or arms, back pain or nerve pain.

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How Pleural Mesothelioma Is Diagnosed

The diagnostic process for pleural mesothelioma begins when a doctor evaluates the initial symptoms of the disease. Chest pain and breathing difficulty warrant a chest X-ray, revealing fluid buildup or tumors around a lung. A primary care physician refers a patient to a specialist when they require further testing.

Specialists must use advanced imaging scans and tissue biopsies to confirm a pleural mesothelioma diagnosis. In addition to identifying cancer, determining the cancer stage and cell type is vital to creating an effective treatment plan.

Most cases of malignant mesothelioma are diagnosed with a high number of epithelial cells, called epithelioid type. The least common cell type is sarcomatoid mesothelioma. It is aggressive and challenging to treat, and most of these cases occur in pleural patients. The biphasic cell type is a blend of these two cell types.

Signs of pleural mesothelioma can aid diagnosis including pleural thickening and pleural effusion
Signs of pleural mesothelioma include fluid buildup and thickening of the lining of the lungs.

Several pleural cancer symptoms and conditions may indicate a person’s history of asbestos exposure and the potential of a mesothelioma diagnosis, including pleural plaques, pleural effusions and pleural thickening.

In a 2020 study published in Case Reports, researchers discovered a case of pleural mesothelioma through testing of metastatic gastric and colonic polyps identified in imaging scans. These polyps were the only sign that the patient had pleural mesothelioma.

Pleural Plaques

Small areas of thickening on the pleura are the most common sign someone has a prior history of asbestos exposure. Pleural plaques are not cancerous and usually do not cause symptoms, but they may indicate an elevated risk for cancer.

Pleural Effusion

Irritation from asbestos fibers can cause excess fluid to build up between the two layers of the pleura. This condition, called pleural effusion, is present in many pleural mesothelioma cases. A little fluid between the pleural layers is healthy. Too much puts pressure on the lungs, causing chest pain that worsens when you cough or take deep breaths.

Pleural Thickening

When large areas of the pleura stiffen because of scarring, it may become difficult and painful to breathe. Pleural thickening around both lungs is often a sign of significant asbestos exposure. Repeated episodes of pleural effusion can cause the pleural thickening to worsen as scar tissue collects.

Pleural Mesothelioma Stages

Pleural mesothelioma staging
Pleural mesothelioma stages

Pleural mesothelioma staging is how doctors determine the progression of cancer in a patient’s body. The stage influences what treatment options doctors may choose to prescribe.

The International Mesothelioma Interest Group created the TNM staging system, the most widely used staging system for pleural mesothelioma.

Stages 1 and 2, which are considered the early stages, indicate localized tumors. Stages 3 and 4, which denote the later stages, classify spreading tumors.

Mesothelioma Life Expectancy by Stage for Patients Eligible for Surgery
Stage Time Progression
Stage 1 22.2 months Tumors remain localized in and around the tissue lining of one lung
Stage 2 20.0 months Cancer cells are entering nearby lymph nodes
Stage 3 17.9 months Cancer has spread to nearby tissues and distant lymph nodes
Stage 4 14.9 months Cancer has spread to distant organs

When doctors diagnose patients in stages 1 or 2, they have more options for treating pleural mesothelioma, including surgery, which offers the best chance at increased survival. In the later stages, treatment options are limited since cancer has begun to spread throughout the body.

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    Pleural Mesothelioma Treatment Options

    Standard pleural mesothelioma treatment options can include surgery, radiation therapy and chemotherapy.

    Chemotherapy is the most common type of pleural treatment. Doctors often prescribe a combination of medicines known as multimodal therapy if they diagnose a patient early in the disease process. These treatments can improve symptoms, such as chest pain and breathing difficulties, and improve survival.

    Patients can access these therapies at top cancer centers across the nation that specialize in pleural mesothelioma treatment.

    5 Tips When Considering Treatment

    1. Set clear treatment goals. What’s most important to you, longevity or quality of life?
    2. Research each treatment option and ask your doctor as many questions as you can think of.
    3. Determine where you will receive recurring treatments such as immunotherapy infusions. Will you have to travel far and how often?
    4. Consider the side effects of suggested chemotherapy drugs and how it may impact your quality of life.
    5. Seek a second opinion. Surgery may still be an option, or you may be eligible for a clinical trial.
    Emily Ward
    Pleural Mesothelioma Survivor

    Chemotherapy

    The most common pleural mesothelioma treatment is chemotherapy. It uses one or more drugs, usually a combination of cisplatin or carboplatin and pemetrexed (Alimta), to kill cancer cells or prevent them from reproducing. Recent advances have improved how well patients respond to chemotherapy, but success rates remain low overall.

    Surgery

    Pleural mesothelioma cancer patients diagnosed in the early stages benefit the most from surgery. The goal of surgery is to remove cancer and prevent it from recurring as long as possible.

    The two most common surgeries for pleural mesothelioma are extrapleural pneumonectomy and pleurectomy and decortication. A pleural mesothelioma cancer specialist can determine if you are eligible for surgery and advise you on which procedure may be the best option for your diagnosis.

    Extrapleural Pneumonectomy: A more aggressive surgical option, the extrapleural pneumonectomy removes the pleura, the entire cancerous lung, the diaphragm and the heart sac (pericardium).

    Pleurectomy and Decortication: A pleurectomy and decortication, or radical pleurectomy, involves removing the tumors and affected pleura (lining of the lung).

    Radiation Therapy

    Targeted radiation can destroy cancer cells and decrease tumor size. Radiation therapy cannot cure pleural mesothelioma, but it is an effective way to manage chest pain. Radiation can also help prevent cancer recurrence after surgery.

    External beam radiation therapy is the most common form of radiation therapy for pleural mesothelioma. Sessions are fast, painless and only take a few minutes.

    Emerging Treatments

    Emerging treatments include novel therapies such as immunotherapy, gene therapy and photodynamic therapy to treat pleural mesothelioma. Researchers are refining these treatments to fight cancer more effectively while causing fewer side effects for the patient.

    You may be able to receive experimental therapy through a pleural mesothelioma clinical trial. Some patients may be eligible for immunotherapy drugs and other emerging treatments through compassionate use programs.

    Top Mesothelioma Doctors in the US

    Doctors who specialize in malignant pleural mesothelioma include oncologists, surgeons and other experts who have years of experience treating this rare disease. 

    Many of these physicians offer unique services such as clinical research trials and mesothelioma support resources. Working with a top mesothelioma specialist provides the best chance at improving prognosis and quality of life.

    Jacques Fontaine
    Thoracic Surgeon

    Dr. Jacques Fontaine is the Director of the Mesothelioma Research and Treatment Center at Moffitt Cancer Center in Tampa, Florida. He specializes in minimally invasive thoracic surgery including robotic surgery. Fontaine became a pleural mesothelioma specialist while studying under Dr. Sugarbaker at the International Mesothelioma Program at Brigham & Women’s Hospital.

    Robert B. Cameron
    Thoracic Surgeon

    Dr. Robert B. Cameron developed a lung-sparing surgery for pleural mesothelioma that not only extends survival but offers greater quality of life by preserving the lung. Cameron’s surgery has a lower risk of complications and studies report longer survival times.

    Abraham Lebenthal
    Thoracic Surgeon

    Dr. Abraham Lebenthal is a respected thoracic surgeon who treats pleural mesothelioma patients at Brigham and Women’s Hospital and Boston VA Hospital. Lebenthal worked alongside Dr. David Sugarbaker at Brigham and teaches at Harvard Medical School.

    Prognosis for Pleural Mesothelioma

    The average pleural mesothelioma prognosis, or the expected course and outcome for the disease, is poor for most patients because this cancer progresses quickly and is resistant to many existing therapies.

    Accurately forecasting an individual’s prognosis is challenging because this disease is complex. Each person responds differently to treatment.

    Pleural Mesothelioma Survival Rate by Year
    Year(s) After Diagnosis Survival Rate
    1 year 73%
    3 years 23%
    5 years 12%
    10 years 4.7%

    Factors That Affect Your Prognosis

    • Stage: The most important factor in a mesothelioma prognosis is the stage of the disease at diagnosis. An early-stage cancer offers a better chance of long-term survival than a late-stage cancer.
    • Cell type: The cell type of mesothelioma tumors also greatly influences prognosis.
    • Age: Younger patients tend to live longer.
    • Gender: Women tend to live longer with the disease than men.
    • Pleural Fluid: Higher amounts of pleural fluid in the chest are associated with a poorer prognosis.
    • Patient Activity Level: More active patients have a better prognosis.
    • Cancer Recurrence: A recurrence of mesothelioma is associated with a poorer prognosis.

    Improving Your Prognosis

    By taking proactive steps, it is possible to live longer and better with malignant pleural mesothelioma. Some patients even achieve remission after treatment and live for years with almost no cancer symptoms.

    The inspiring stories of mesothelioma survivors reveal many ways to confront the challenge of cancer. However, many survivors make the same basic choices to promote their well-being.

    7-year pleural mesothelioma survivor Emily Ward
    I belong to a group of cancer patients who live with a grim prognosis. I know I have no cure — yet — but am encouraged with every new study, mesothelioma treatment approval and inspiring survivor story.
    Emily Ward
    Pleural mesothelioma survivor

    Tips for Living Longer with Pleural Mesothelioma

    Following the advice of your physician and taking steps toward a healthy lifestyle, such as exercise and a mesothelioma diet, can help improve your prognosis and extend life expectancy.

    • Seek treatment from an experienced mesothelioma specialist
    • Improve your lifestyle to boost your overall health
    • Access the latest therapies through a clinical trial
    • Use palliative and complementary medicine to improve your quality of life

    Common Questions About Pleural Mesothelioma

    How does asbestos affect the lungs?

    Damage to asbestos-containing materials releases asbestos fibers as dust. When inhaled, these fibers become trapped in the pleural lining of the lungs. The asbestos fibers trigger an inflammatory response by the immune system. Over the course of decades, this process leads to tissue scarring and DNA damage that causes mesothelioma.

    What are the symptoms of pleural mesothelioma?

    The symptoms of pleural mesothelioma include shortness of breath, chest pain, dry cough, wheezing, respiratory complications, fever and night sweats. Other symptoms may develop as the disease progresses.

    Is pleural mesothelioma curable?

    Pleural mesothelioma is incurable at all four stages. Clinical trials are providing hope for a cure as they test innovative treatments including immunotherapy and gene therapy.

    How long do pleural mesothelioma patients live?

    Most pleural mesothelioma patients have a life expectancy of one year. Patients who are diagnosed early and qualify for aggressive treatment live an average of 22.2 months, and some live for many years.


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