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What Is Pleural Mesothelioma?

Malignant pleural mesothelioma is a rare type of 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, leading to the disease.

Pleural mesothelioma is the most common type of mesothelioma cancer, accounting for 70% to 79% of mesothelioma cases. Doctors diagnose more than 3,000 new patients with pleural mesothelioma every year in the United States.

Pleural mesothelioma forming around a lung after asbestos exposure
How pleural mesothelioma develops

What Causes Pleural Mesothelioma?

The primary cause of pleural mesothelioma is asbestos exposure.

Mesothelioma develops when a person inhales asbestos and the fibers get lodged in the lung lining. 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, which mutate healthy pleural cells into cancerous cells.

Who Is At Risk?

Occupational exposure to asbestos is the No. 1 cause of pleural mesothelioma, and there are several occupations that pose a high risk.

It usually takes from 20 to 60 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 70.

  • Military Veterans: The U.S. military used asbestos widely in machinery and construction materials. Veterans in shipyards were among the most heavily exposed.
  • Firefighters: In addition to exposure to asbestos in burning buildings, firefighting equipment, including ventilators and masks, were made with asbestos.
  • Construction Workers: No industry used asbestos more than the construction industry. As a result, construction workers are among occupations most at risk of pleural mesothelioma.
  • Power Plant Workers: Asbestos was used in arch chutes, electrical insulation, electrical panels and more, exposing many power plant workers throughout the U.S.
  • Industrial Workers: Those who worked at industrial worksites and applied industrial insulation were heavily exposed to asbestos.

These workers often unknowingly brought asbestos fibers home on their clothing and tools, which led to secondary exposure among their loved ones. 

Environmental exposure is a less common pathway of exposure, but it can affect entire communities. For example, the town of Libby, Montana, recently cleaned up asbestos contamination resulting from an asbestos-contaminated vermiculite mining operation.

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Symptoms of Pleural Mesothelioma

People with pleural mesothelioma have symptoms that make it difficult to breathe. Some patients also develop swelling of the face or arms, back pain or nerve pain.

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

For many people, mesothelioma symptoms are not noticeable until the cancer is in a later stage. Patients rarely mention weight loss and fatigue during their initial doctor visit. These symptoms may only appear if the cancer is advanced.

Several pleural cancer symptoms may indicate a person’s history of asbestos exposure and the potential of a mesothelioma diagnosis. According to many years of research on people exposed to asbestos, these conditions include 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 of 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 develops.

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. Asbestosis patients remain more at risk of developing asbestos-related lung cancer rather than pleural mesothelioma, but the latter has been documented in medical literature.

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, which can reveal fluid buildup or tumors around a lung.

A primary care physician refers a patient to a specialist when they require further testing. A biopsy is the most important diagnostic test for mesothelioma because it can detect cancerous tissue.

Diagnostic tools include:
  • Physical Examination: During a physical examination a doctor will look for abnormalities, such as a bump on the skin, and check parts of the body that feel tender or painful.
  • Imaging Scans: X-rays, MRIs and PET scans help spot the location of tumors.
  • Blood Tests: Blood tests identify biomarkers of cancer but cannot diagnose mesothelioma alone.
  • Biopsy: Biopsies are tissue samples that can confirm a cancer diagnosis.

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

Cell type is important in determining treatment plans. The three different cell types that can make up pleural mesothelioma tumors are identified with a biopsy. 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.

Staging

Pleural mesothelioma staging is how doctors determine the progression of cancer in a patient’s body. The stage influences what treatment options doctors 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
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Pleural Mesothelioma Treatment Options

Standard pleural mesothelioma treatment options can include surgery, radiation therapy and chemotherapy. Doctors often prescribe a combination of these therapies, known as multimodal therapy, if they diagnose a patient early in the disease process.

Patients can access these therapies at top cancer centers across the nation that specialize in pleural mesothelioma treatment. These treatments can improve symptoms, such as chest pain and breathing difficulties, and improve survival.

  • Surgery: Surgery is used to diagnose this cancer, relieve symptoms and remove tumors. Early-stage patients benefit the most from tumor-removing surgery. Procedures include extrapleural pneumonectomy and pleurectomy and decortication.
  • Chemotherapy: Chemotherapy is the most common type of pleural treatment. 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.
  • Radiation: 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.
  • Immunotherapy: The U.S. Food and Drug Administration approved the Opdivo and Yervoy combination immunotherapy for pleural mesothelioma in 2020. Patients not eligible for surgery have access to these drugs, also known as nivolumab and ipilimumab, respectively.
  • Emerging Treatments: Emerging treatments for pleural mesothelioma include novel therapies such as gene therapy and photodynamic therapy. 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.

Palliative care doctors prescribe medications and therapies to control pleural mesothelioma symptoms and improve quality of life at all stages.

“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

Top Pleural Specialists in the U.S.

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 the Dana-Farber Cancer Institute. Lebenthal also teaches at Harvard Medical School.
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Pleural Mesothelioma Prognosis and Survival Rates

The overall pleural mesothelioma prognosis is poor, with a median survival of eight to 14 months. The five-year survival rate is 20% for early-stage cases with localized tumors and 12% for all stages combined. Some patients have lived longer than a decade with ongoing treatment.

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

Pleural mesothelioma prognostic factors range from personal factors, such as your age and activity level, to factors related to the cancer, such as stage and cell type.

  • 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.

Certain biomarkers may also be associated with patient survival, including calcium and platelet count. Talk to your medical team about how these factors may impact your personal 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 several ways to confront the challenge of cancer. However, many survivors make the same basic choices to promote their well-being.

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.

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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