Pleural Mesothelioma

Pleural mesothelioma is a malignant cancer that develops on the lining of the lungs called the pleura. It is the most common type of mesothelioma. Although the prognosis is typically poor, finding a pleural cancer specialist can diversify your treatment options and help improve your prognosis.

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Pleural mesothelioma accounts for about 75 percent of all mesothelioma cases. Like other types of mesothelioma, this particular form of the disease gets its name because of where it is formed — in the pleura, a soft tissue that surrounds the lungs. In almost all cases, pleural mesothelioma is caused by asbestos exposure.

The first symptoms of pleural mesothelioma typically include chest pain and shortness of breath. You may experience no symptoms at all 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. It often takes decades (20 to 50 years) for mesothelioma to develop after someone is first exposed to asbestos. This lag time — called a latency period — explains why the disease usually affects older people.

How Asbestos Leads to Mesothelioma

Once inhaled into the lungs, asbestos travels to the pleura. The body then struggles to get rid of the needle-like fibers. Over a long period of time, trapped fibers irritate the pleural membrane, causing chronic inflammation and scarring.

Odds of Development

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

Who Is Affected?

About 80 percent of people diagnosed with pleural mesothelioma are older men, mainly because most asbestos exposure occurred at industrial jobs traditionally worked by men.

What Are Pleural Mesothelioma Symptoms?

Symptoms of pleural mesothelioma include persistent dry or raspy cough, coughing up blood (hemoptysis), shortness of breath (dyspnea), and difficulty swallowing (dysphagia). There are four stages of mesothelioma that doctors use to describe how far the cancer has progressed. For many people, unfortunately, symptoms are not noticeable until the cancer is in a later stage — stage III or IV.

Asbestos fibers can cause excess fluid to build up between the two layers of the pleura, a condition called pleural effusion. While a little fluid in your pleural space is important, too much can make breathing difficult. The extra fluid puts pressure on the lungs, causing chest pain that gets worse when you cough or take deep breaths.

Persistent dry or raspy cough

Coughing up blood (hemoptysis)

Shortness of breath (dyspnea)

Pain in the lower back or rib area

Painful breathing

Lumps under the skin on the chest

Difficulty with swallowing (dysphagia)

Night sweats or fever

Unexplained weight loss

Fatigue

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.

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Diagnosis

Two layers of tissue make up the pleura. These tissues protect and support the lungs and other important structures of the chest. They also produce lubricating fluid between to help the lungs move smoothly as we breathe. The outer layer, the parietal pleura, lines the entire inside of the chest cavity. The inner layer, or visceral pleura, covers the lungs.
Mesothelioma commonly affects both layers of the pleura. The cancer generally forms in one layer of the pleura and rapidly invades the other pleural layer, diaphragm, chest wall or lung. If the cancer reaches nearby glands called lymph nodes, it can metastasize, or spread, to other parts of the body.
Non-cancerous conditions like pleural plaques and atelactasis can develop on the surface of the pleura as a result of asbestos exposure and these conditions are not associated with pleural mesothelioma. They don’t develop into cancer and they don’t increase the risk of developing lung cancer or pleural mesothelioma.

Diagram of Healthy Lung vs Diseased Lung

Pleural mesothelioma can be difficult to diagnose, since symptoms usually do not arise until long after the first exposure to asbestos. Since many diseases of the lungs and respiratory system have the same symptoms as pleural mesothelioma, doctors may mistake it for the flu or pneumonia.

Difficult to Confirm a Diagnosis

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

Reliable Ways to Diagnose

More reliable ways to diagnose the disease include thoracoscopy, which allows doctors to view the patient's chest through a small camera and collect a tissue biopsy, which doctors use to test tissue and fluid samples for cancerous cells.

Diagnostic Imaging

To achieve the best treatment options and survival outlook, an early diagnosis is crucial. 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.

Chest X-rays

X-rays can reveal pleural effusions and pleural-based masses in some cases.

CT Scans

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

PET Scans

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

With the introduction of the PET scan in the early 1960s, doctors could better distinguish between cancerous and non-cancerous growths on the pleura. While imaging plays an important role in staging mesothelioma and guiding treatment, it cannot be used to diagnose the cancer on its own.

Cytology and Biopsy

Other tests are needed to diagnose mesothelioma with a high level of confidence. One test is thoracentesis, in which doctors insert a hollow needle into the lungs to collect pleural fluid.
Doctors look at the cell samples from the fluid under a microscope. They look for substances called biomarkers that indicate the presence of cancer. Specialists can use this analysis to make an accurate diagnosis in 80 percent of malignant mesothelioma cases.

Cytologies and biopsies help to accurately diagnose mesothelioma.

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. During a biopsy, doctors remove a tissue sample and look for cancerous cells. 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.
Similar diagnostic tests will be performed on different parts of the body for other forms of mesothelioma. For example, even though similar imaging scans and biopsies are used to diagnose all types of mesothelioma, the part of the body that undergoes imaging or biopsy will differ for each type of mesothelioma.
Because mesothelioma locally invades the body cavity in which it develops before spreading, doctors rarely have a hard time differentiating one form of mesothelioma from another. Only in late stage IV cases may there be enough spreading to or from the lung to the abdomen to question whether the cancer originated in the lining of the lungs or abdomen.

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Treatment Options

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Historically, doctors have treated pleural mesothelioma with traditional cancer treatments like surgery, chemotherapy and radiation therapy. Most treatment plans used a combination of the three which is called multimodal therapy.
Depending on how far your mesothelioma has progressed, these therapies can be either either cytoreductive (meaning reduction of tumor cells) or palliative (meaning control of symptoms).
Cytoreductive treatments aim to control the cancer, while palliative treatments only address the symptoms and try to improve the quality of life for patients.

The most common treatments for pleural mesothelioma are the following:

Surgery
Chemotherapy
Radiation
Mulitmodal

Surgery

Surgery may be a treatment option for 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, a protective membrane that covers the heart.
Chemotherapy

One of the most common mesothelioma treatments is chemotherapy, which uses one or more drugs to kill cancer cells and decrease the size of tumors. While recent advancements have improved how well patients respond to chemotherapy, success rates have been low overall.
However, clinical trials that combine several chemotherapy drugs in one treatment have shown great potential. 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.
Radiation Therapy

During radiation therapy, doctors administer targeted radiation that destroys cancer cells and decreases tumor size. Pleural mesothelioma often resists radiation therapy, but it can be effective for managing symptoms like 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.
Multimodal Therapies

While there is no single course of treatment for pleural mesothelioma that all doctors agree on, the majority of experts believe that survival is best when several treatments are used. This approach is known as multimodal therapy. 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 pleural mesothelioma 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.

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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 pleural mesothelioma, they can ease cancer symptoms and reduce side effects of cancer treatment.
For example, certain herbs like slippery elm bark may help relieve a cough and pulmonary discomfort. Some yoga breathing techniques may feel soothing to the lungs and most offer a sense of relaxation to ease stress. Mental health counseling is the most effective therapy for psychological stress.
Acupuncture and acupressure are clinically proven to help control nausea and vomiting caused by chemotherapy. Other complementary therapies used in cancer care include:

Nutrition
Meditation
Tai chi and qigong
Osteopathic medicine

Prognosis

After the diagnostic process, doctors analyze the expected course and outcome for the disease — your prognosis. While some cancers carry promising prognoses, malignant mesotheliomas do not. Providing an accurate prognosis challenges doctors because the disease is so complex and aggressive.

Stage of disease at diagnosis
Age of patient
Gender of patient
Patient's smoking history
Painful breathing

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 change 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, which is also a significant factor for survival.

Other important prognostic factors include your age, sex and smoking history. Generally speaking, survival rates are higher for female patients, non-smokers and patients younger than 55. A cancerous tumor's cellular makeup — called histology — also can have a major influence on prognosis.

Subtypes

Among the subtypes of this disease, epithelial mesothelioma makes up the most cases (50 to 70 percent) and also offers the most long-term hope.

40% Survive one year after diagnosis.
20% Survive two or more years after diagnosis.
10% Survive three years after diagnosis.

Patients with epithelial mesothelioma respond best to treatment and typically live at least one year. Other subtypes like sarcomatoid and biphasic mesothelioma have poorer 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 like chemotherapy. Because biphasic mesothelioma is a mix of both epithelial and sarcomatoid cells, how a patient responds to treatment and how long they live will depend upon the percentages of epithelial and sarcomatoid cells. A higher ratio of epithelial cells translates into longer survival.

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Author

Karen Selby

Karen Selby is a registered nurse and a Patient Advocate at The Mesothelioma Center. She worked in several subspecialties within nursing before joining Asbestos.com in 2009.