Pleural mesothelioma, the most common type of asbestos-related cancer, forms on the lining of the lungs. Symptoms tend to appear in the late stages of the disease.Get a Free Pleural Mesothelioma Guide
It is named for the location where it forms — in the pleura, a soft tissue that covers the lungs.
Pleural mesothelioma (PM) accounts for almost 75 percent of all cases of mesothelioma, a relatively rare cancer caused by asbestos exposure.
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Initial 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. 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.
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.Learn more about how asbestos causes pleural mesothelioma
Symptoms of pleural mesothelioma include shortness of breath, chest pain or pleura pain, persistent dry or raspy cough, coughing up blood and difficulty swallowing.
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.
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.
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.
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.
X-rays reveal pleural effusions and pleural tumors in some cases.
More advanced CT scan images often show evidence of asbestos exposure.
PET scans can detect signs of cancerous spread to the lymph nodes.
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.
With the introduction of the PET scan in the early 1960s, doctors could better distinguish between cancerous and noncancerous growths on the pleura. While imaging plays an important role in staging malignant mesothelioma and guiding treatment, it cannot be used to diagnose the cancer on its own.
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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.
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 to look 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.
Similar diagnostic tests performed on different parts of the body help diagnose other forms of mesothelioma. For example, even though similar 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.
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 stage IV cases is there enough spreading to or from the lung to the abdomen to question whether the cancer originated in the lining of the lungs or abdomen.Learn more about mesothelioma diagnosis
Several different staging systems exist for malignant pleural mesothelioma. All of them define four stages of progressive development. The first two stages indicate localized tumors. The last two stages classify spreading tumors.
The International Mesothelioma Interest Group (IMIG) 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
Historically, doctors have treated pleural mesothelioma with traditional cancer treatments such as surgery, chemotherapy and radiation therapy. Most treatment plans combine the three, which is called multimodal therapy.
Depending on how far your cancer has progressed, these therapies can be either cytoreductive (reduction of tumor cells) or palliative (control of symptoms).
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.
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 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 therapy
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
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.Learn more about chemotherapy
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 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.
Whether you are concerned about finding the right doctor or treatment center for your pleural mesothelioma diagnosis, we offer several programs to guide you or your loved ones.
Our Doctor Match program not only locates a doctor near you but also a specialty cancer center. We also research the best clinical trials exploring how to treat the disease with the latest experimental 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 the body and mind.
Mind-body therapies stimulate 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, anxiety and depression.
Nutrition and herbalism help the immune system fight infection, promote quicker recovery, maintain energy and boost mood. For example, protein fuels recovery; and certain herbs, such as slippery elm bark, may help relieve cough and pulmonary discomfort.
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 help control nausea and vomiting caused by chemotherapy.
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. Patients with the most common cell subtype, called epithelial, live an average 200 days longer than patients with the least common subtype.
Patients with epithelial mesothelioma 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 longer survival.
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.
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