Written By: Karen Selby, RN,
Last modified: November 2, 2021

What Is Pleural Mesothelioma?

Malignant pleural mesothelioma is a type of rare cancer that develops in the pleura, the thin membrane that 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. 

Doctors diagnose roughly 3,000 new patients with pleural mesothelioma every year in the United States.

Where pleural mesothelioma forms
Where pleural mesothelioma forms

Pleural Mesothelioma Facts

  • Pleural mesothelioma is the most common type of mesothelioma cancer.
  • Symptoms of pleural mesothelioma include shortness of breath, chest pain, dry cough, wheezing and fatigue.
  • Pleural mesothelioma is not curable. It is managed with cancer treatment, which can improve life expectancy and alleviate symptoms.
  • The average life expectancy with pleural mesothelioma is four to 18 months, and the five-year survival rate is 10%.

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

Pleural Plaques

Small areas of thickening on the pleura are the most common sign someone has a 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.

Asbestosis

Asbestosis is a pulmonary disease marked by progressive scarring of lung tissue caused by asbestos exposure. This condition does not affect the pleural lining. There is a greater risk for someone diagnosed with asbestosis to develop asbestos-related lung cancer rather than pleural mesothelioma, but the latter has been documented in medical literature.

With this disease, finding a specialist is critical. You need someone who really understands it and knows how to treat it aggressively to get the best possible outcome.
Dr. Jacques Fontaine
Pleural mesothelioma surgeon

What Causes Pleural Mesothelioma?

The cause of pleural mesothelioma is exposure to asbestos fibers, which a person can inhale into the lungs. 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. 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.

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.

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.

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Symptoms of Pleural Mesothelioma

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

Pleural mesothelioma 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
  • Fluid in the lungs

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.

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.

Cell Types

Tissue biopsies identify the three different cell types that can make up pleural mesothelioma tumors, and this information is important in determining treatment plans. Most cases of malignant mesothelioma are diagnosed with a high number of epithelial cells, also called epithelioid cells. This cell type responds best to cancer treatment.

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. How it responds to treatment depends on the ratio of cells. If more epithelioid cells are present, it will respond better than tumors composed of more sarcomatoid cells.

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.

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    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.

    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.

    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

    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.

    A new method of delivering chemotherapy known as heated intrathoracic chemotherapy, also called HITHOC, is applied directly to the pleural cavity following surgery. The chemotherapy drugs are heated before being circulated throughout the pleural cavity.

    Surgery

    Surgery is used to diagnose the cancer, relieve symptoms and remove tumors. A common diagnostic surgery includes thoracoscopy, which is a minimally invasive procedure to collect tumor tissue samples for biopsy testing.

    Patients diagnosed with later stages of disease may benefit from therapeutic surgeries to control symptoms. A thoracentesis is a minimally invasive surgery that removes fluid from the pleural cavity through a tube inserted through small incision in the chest. A pleurodesis also removes pleural fluid, but it takes the procedure a step further by eliminating the pleural space by fusing the two layers of the pleura together. This prevents the recurrence of fluid buildup known as pleural effusion.

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

    The two most common tumor-removing 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, also known as a P/D 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.

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    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.

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

    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

    Hope for a Mesothelioma Cure

    There is no definitive cure for pleural mesothelioma. However, a combination of surgery, chemotherapy and radiation therapy have allowed patients to improve their prognosis. Even patients who are not eligible for surgery have survived for years after their diagnosis thanks to experimental clinical trials and emerging treatments such as immunotherapy.

    Researchers around the world have dedicated their careers to finding more effective treatments with the goal of curing pleural mesothelioma and diagnosing the cancer at an earlier stage.

    • Emily Ward was diagnosed in 2012 after working for 43 years as a registered nurse. She put her medical background to work and found the top mesothelioma specialists available to treat her cancer, including the late Dr. David Sugarbaker. She underwent a pleurectomy and decortication, three rounds of chemotherapy and immunotherapy with Keytruda, Opdivo and Yervoy. Ward is enjoying a good quality of life doing many of the things she did before her diagnosis.
    • Tim Crisler passed the 17-year mark as a pleural mesothelioma survivor in 2019. He credits his lung-removing extrapleural pneumonectomy surgery for his long-term survival. He still deals with residual pain, but he has been able to travel the country on his beloved Harley-Davidson since his diagnosis in 2002. “I have absolutely no issues with mesothelioma cancer today,” he said. “That hasn’t touched me in a long time.”
    • Sallie Morton survived pleural mesothelioma for four years while foregoing conventional treatment. She wasn’t eligible for surgery when she was diagnosed in 2013 at the age of 87, and she declined chemotherapy after seeing too many friends deal with the harsh side effects of the drugs. She did it her way, surprising doubters and hosting events and parties to the end. “She’s one gutsy broad,” is how she often described herself.

    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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