Pleural Mesothelioma

Pleural mesothelioma is a cancer that develops on the lining of the lungs. It is the most common type of asbestos-related cancer. Specialists use the latest therapies to treat it, and clinical trials offer access to new treatments such as immunotherapy.

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Pleural mesothelioma (PM) accounts for almost 75 percent of all cases of the rare cancer caused by asbestos exposure. Like other types of mesothelioma, it is named for the location where it forms — in the pleura, a soft tissue that covers the lungs.

Initial symptoms of pleural mesothelioma typically include chest pain and shortness of breath. You may experience no symptoms in the first few stages of the cancer’s progression.

The life expectancy of someone with pleural mesothelioma is less than 18 months, but some patients live much longer. Combining several treatments, such as surgery, chemotherapy and radiation therapy, helps people live longer with PM.

It often takes 20 to 50 years for mesothelioma to develop after first exposure to asbestos. This lag time — called a latency period — explains why the disease usually affects older people.

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How Does Asbestos Cause Pleural Mesothelioma?

After a person inhales airborne asbestos, the human body struggles to remove the mineral's needle-like fibers from the lungs. Over a long period of time, trapped fibers migrate to the pleural lining and cause irritation, chronic inflammation and genetic damage.

In 2 to 10 percent of people heavily exposed to asbestos, the trapped fibers cause pleural mesothelioma by triggering genetic changes that turn cells cancerous. These cancerous cells grow fast and uncontrollably, forming tumors that wrap around the lungs.

Almost 80 percent of people diagnosed with pleural mesothelioma are men older than 75. Asbestos exposure most commonly occurred at industrial jobs where men historically comprised the majority of the workforce in the U.S.

Learn more about how asbestos causes pleural mesothelioma

What Are the Symptoms of Pleural Mesothelioma?

Symptoms of pleural mesothelioma include shortness of breath (dyspnea), chest pain or pleura pain, persistent dry or raspy cough, coughing blood (hemoptysis) and difficulty swallowing (dysphagia).

Three in four pleural mesothelioma patients experience shortness of breath and more than half report chest pain.

There are four stages of mesothelioma that doctors use to describe cancer progression. For many people, unfortunately, symptoms are not noticeable until the cancer is in a later stage — stage III or IV.

In their first meeting with a doctor, a majority of pleural mesothelioma patients report chest pain and shortness of breath. Patients rarely mention weight loss and fatigue during their initial doctor visit, but these symptoms may be present if the cancer is in a later stage. Some patients develop swelling of the face or arms, back pain and nerve pain.

Pleural Mesothelioma Symptoms

  • Persistent dry or raspy cough
  • Coughing blood
  • Shortness of breath
  • Pain in the lower back or rib area
  • Painful breathing
  • Lumps under the skin on the chest
  • Difficulty swallowing
  • Night sweats or fever
  • Unexplained weight loss
  • Fatigue
Pleura of the lungs

Asbestos fibers can cause excess fluid to build between the two layers of the pleura, a condition called pleural effusion that’s present in 90 percent of pleural mesothelioma cases. While a little fluid is important, too much makes breathing difficult. The extra fluid puts pressure on the lungs, causing chest pain that worsens when you cough or take deep breaths.

Two layers make up the lung pleura. The outer layer, called the parietal pleural membrane, lines the entire inside of the chest cavity. The inner layer, or visceral pleural membrane, covers the lungs. A pleural mass can develop on either layer and quickly spread to the other layer. As tumors develop on the pleural surface, they grow to form a sheath-like mass around the lung.

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Pleural mesothelioma is sometimes difficult to diagnose. Because many diseases of the lungs and respiratory system resemble symptoms of PM, doctors may mistake it for the flu or pneumonia without extensive testing.

The diagnostic process begins when a doctor, often a primary care physician, evaluates initial symptoms. Chest pain and breathing impairment warrant a chest X-ray, typically the first test to show fluid or tumors around the lungs.

Referral to a pulmonologist, oncologist or general hospital is common after an abnormal X-ray. Further imaging, blood tests and tissue biopsies are used to confirm a pleural mesothelioma diagnosis.

It is challenging for doctors to tell the difference between some cases of pleural mesothelioma and lung cancer. While doctors may suspect mesothelioma based on a patient's symptoms, history of asbestos exposure and abnormal imaging scan results, these signs are not enough to confirm a diagnosis.

The most reliable tool to diagnose the disease is a thoracoscopy. This minimally invasive procedure allows doctors to view the patient's chest through a small camera and collect a tissue sample, also known as a biopsy. A pathologist will evaluate the biopsy, distinguish the cancer’s cell type and confirm the diagnosis.

Diagnostic Imaging

After a review of medical and occupational history and a physical examination, patients typically undergo imaging tests that can reveal potentially cancerous tumors. There are three primary imaging tests used to diagnose pleural mesothelioma: Chest X-rays, CT scans and PET scans.

  • Two people looking at an X-ray

    Chest X-rays

    X-rays reveal pleural effusions and pleural tumors in some cases.

  • Person getting a CT Scan

    CT Scans

    CT scans provide more advanced images that often show evidence of asbestos exposure.

  • Person getting a PET scan

    PET Scans

    PET scans can detect signs of cancerous spread to the lymph nodes.

Medical oncologist Dr. Tirrell Johnson explains pathology and the important role it plays in mesothelioma cancer progression.

How Does Cytology and a Biopsy Confirm Diagnosis?

Other tests are needed to diagnose mesothelioma with a high level of confidence. One test is a thoracentesis, in which doctors insert a hollow needle into the lungs to collect pleural fluid. This test is used when patients exhibit pleural effusions. The sample undergoes cytology testing to look for cancer cells in lung fluid.

Pathologists view fluid samples under a microscope for substances called biomarkers that indicate the presence of cancer. A thoracentesis provides an accurate diagnosis in 80 percent of malignant pleural mesothelioma cases.

Sometimes, however, a thoracentesis isn't enough, or there may be no pleural fluid to study. In these cases, doctors perform biopsies to collect pleural tissue samples. A thoracoscopic biopsy produces an accurate diagnosis in 98 percent of mesothelioma patients.

If a series of tests or biopsies confirm the presence of mesothelioma, doctors develop a treatment plan based on the results of those evaluations.

Similar diagnostic tests performed on different parts of the body help diagnose other forms of the cancer. For example, even though imaging scans and biopsies diagnose all types of mesothelioma, the part of the body that undergoes imaging or biopsy differs for each type of mesothelioma cancer.

Because mesothelioma locally invades the body cavity in which it develops before spreading, doctors rarely struggle to differentiate one form of mesothelioma from another. Only in late-stage IV cases is there enough spreading to or from the lung and to the abdomen to question whether the cancer originated in the lining of the lungs or abdomen.

Learn more about mesothelioma diagnosis

Who Are the Best Pleural Mesothelioma Specialists?

Several doctors across the nation specialize in the treatment of the asbestos-related cancer. Dr. David Sugarbaker, known as "Mr. Mesothelioma" by his patients and peers, is the director of the Lung Institute at Baylor College of Medicine. He is a pioneer in the treatment of mesothelioma.

Dr. Abraham Lebenthal at Brigham and Women's Hospital and the VA Boston Healthcare System specializes in treating lung cancer and mesothelioma, and he's spent years working with U.S. veterans diagnosed with the asbestos-related cancer.

Another well-known thoracic surgeon who has helped hundreds of pleural mesothelioma patients is Dr. Robert Cameron at UCLA Jonsson Comprehensive Cancer Center. He also plays a crucial role in the cancer treatment of veterans at the VA Greater Los Angeles Healthcare System.

David Sugarbaker M.D.
Dr. David Sugarbaker Director of Lung Institute at Baylor College of Medicine, Chief of General Thoracic Surgery

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Pleural Mesothelioma Stages Diagram

How Is Pleural Mesothelioma Staged?

Several different staging systems exist for malignant pleural mesothelioma. All of them define four stages of progressive development. These are usually labeled I, II, III and IV. The first two stages indicate localized tumors, and the last two stages classify spreading tumors.

The International Mesothelioma Interest Group created the most widely used staging system for pleural mesothelioma. It applies the tumor-node-metastasis (TNM) approach used to stage many other cancers.

Two other staging systems are used less often, including the Brigham and Women’s staging system and the Butchart staging system. Pleural mesothelioma specialist Dr. David Sugarbaker created the Brigham and Women’s staging system based upon his extensive surgical experience. Butchart, developed by Eric Butchart in 1976, is the oldest staging system.

Learn more about pleural mesothelioma staging

What Are the Treatment Options for Pleural Mesothelioma?

Historically, doctors have treated pleural mesothelioma with traditional cancer treatments such as surgery, chemotherapy and radiation therapy. Most treatment plans combined the three, which is called multimodal therapy.

Depending on how far your cancer has progressed, these therapies can be cytoreductive or palliative.

Cytoreductive treatments aim to control the cancer, while palliative treatments address symptoms and try to improve quality of life for patients. Certain palliative treatments, such as chemotherapy to improve pulmonary symptoms and radiation therapy to reduce chest pain, also help control cancer growth.

  • Multimodal Therapy

    While there is no single course of treatment for pleural mesothelioma that all doctors agree on, experts believe the best chance of survival is achieved with a combination of treatments. This approach to cancer treatment is known as multimodal therapy.

    It takes a multidisciplinary team to treat someone with pleural mesothelioma. Surgeons spearhead minor and major surgical procedures, while radiologists administer radiation therapy, and traditional oncologists oversee chemotherapy.

    Researchers are constantly experimenting with surgery, chemotherapy and radiation therapy to uncover the most effective combination and timing for these treatments.

    In one example of a multimodal PM treatment, doctors first perform extrapleural pneumonectomy surgery. Radiation therapy is then administered to reduce the risk of local cancer recurrence, and distant spread is prevented with chemotherapy.

    Learn more about multimodal treatment
  • Surgery

    Surgery is a treatment option for early-stage pleural mesothelioma patients. One common procedure is pleurectomy/decortication (P/D), in which doctors remove the tumor and affected pleura. A more aggressive surgical option, extrapleural pneumonectomy (EPP), aims to remove the pleura, the entire cancerous lung, the diaphragm and the pericardium, which is a protective membrane that covers the heart.

    Learn more about surgery
  • Chemotherapy

    The most common mesothelioma treatment is chemotherapy, which uses one or more drugs to kill cancer cells to decrease the size of tumors. While recent advancements improve how well patients respond to chemotherapy, success rates remain low overall.

    A combination of cisplatin and pemetrexed (Alimta) is the most effective chemotherapy regimen for pleural mesothelioma. Other effective chemotherapeutic drugs include gemcitabine, carboplatin and doxorubicin.

    While researchers have not yet found a combination of chemotherapy drugs that can cure pleural mesothelioma, this is a primary goal of countless clinical trials.

    Learn more about chemotherapy
  • Radiation Therapy

    During radiation therapy, doctors administer targeted radiation that destroys cancer cells and decreases tumor size. Radiation therapy cannot cure pleural mesothelioma, but it effectively manages chest pain.

    One of the most successful radiotherapy techniques is called intensity-modulated radiotherapy. Usually given in intervals after a radical surgery like EPP, this technique prevents cancer recurrence in many cases.

    Learn more about radiation therapy
  • Targeted Therapy

    Targeted therapies, such as immunotherapy and gene therapy, focus on cancer cells directly without harming healthy cells. A targeted approach causes fewer side effects because healthy cells remain unharmed. These therapies are accessible by enrolling in a clinical trial or seeing a mesothelioma specialist.

Complementary Therapies

Some people with pleural mesothelioma take an integrative approach to treatment by combining complementary therapies with traditional cancer treatment. While complementary therapies cannot cure malignant pleural mesothelioma, they ease cancer symptoms and reduce side effects of treatment.

Different types of complementary therapies promote healing in body and mind.

Body-based complementary therapies support function and health of bones, joints, muscles and lymphatic and blood systems. For example, acupuncture and acupressure are clinically proven to control nausea and vomiting caused by chemotherapy drugs.

Mind-body therapies stimulate the connection between the body and mind and teach people ways to control pain, relax and release stress. Some yoga breathing techniques feel soothing to the lungs, and most offer a sense of relaxation to ease stress. Meditation helps people cope with uncertainty. It also helps reduce anxiety and ease depression.

Nutrition and herbalism help the immune system fight infection, promote quicker recovery, maintain energy and boost mood. For example, protein fuels recovery. Certain herbs, such as slippery elm bark, may help relieve cough and pulmonary discomfort.

Mental health counseling is the most effective therapy for psychological stress. Joining a support group allows patients to connect and get support from other people facing cancer. Our online mesothelioma support group meets the second Wednesday of every month at 8 p.m. EST.

Learn more about complementary therapies for pleural mesothelioma

Prognosis

Throughout the diagnostic process, doctors analyze the expected course and outcome for the disease — known as your prognosis. Providing an accurate prognosis challenges doctors because the disease is so complex, and each person responds differently to treatment.

The most important factor in a pleural mesothelioma prognosis is the stage of the disease at diagnosis. Staging is how doctors describe cancer's progression. An early-stage cancer offers a better chance of long-term survival than a late-stage cancer.

Patients in otherwise good health who can still perform normal daily tasks tend to respond better to treatment — another significant factor for survival.

The makeup, or histology, of a mesothelioma cancer cell greatly influences prognosis.

Among the subtypes of this disease, epithelial mesothelioma represents the majority of cases and is associated with the longest survival. Patients with this type respond best to treatment and typically live at least one year. Those with sarcomatoid and biphasic subtypes of mesothelioma have shorter survival rates, usually around six or eight months.

Sarcomatoid cells are the most aggressive mesothelioma cells in terms of rapid growth and resistance to treatments such as chemotherapy. Biphasic mesothelioma is a mix of epithelial and sarcomatoid cells. The ratio of both cells determines how a biphasic patient responds to treatment and how long they live. A higher ratio of epithelial cells translates into a longer survival time for the patient.

Other important prognostic factors include your age, sex and smoking history. Generally speaking, survival rates are higher for female patients, nonsmokers and patients younger than 55.

If doctors find cancer in fluid around the lungs, life expectancy is impacted. Cancerous pleural effusions are associated with shorter survival. Treatment with surgery or chemotherapy may extend survival for these patients.

Factors That Affect Your Prognosis

  • Stage of disease at diagnosis
  • Cell subtype
  • Age of patient
  • Gender of patient
  • Patient's smoking history
  • Amount of fluid in the chest
  • Patient’s activity level
  • Cancer recurrence

Pleural Mesothelioma Survival Rate by Year

  • 40% graph icon One year after diagnosis
  • 20% graph icon Two years after diagnosis
  • 10% graph icon Three or more years after diagnosis

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