Pleural mesothelioma is a cancer that develops in the cells that form the outer lining of the lungs and inner lining of the chest cavities. 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.
Mesothelioma is the rare cancer caused by asbestos exposure, and almost 75 percent of all cases form in the pleura, the soft tissue that covers the lungs. Pleural mesothelioma is named for the location where it forms, like the other three types of mesothelioma.
It appears that it usually takes from 20 to 50 years for mesothelioma to develop after a person’s first exposure to asbestos. This lag time — called a latency period — explains why the disease usually affects older people.
Most people diagnosed with pleural mesothelioma are men older than 75. Asbestos exposure historically occurred in professions where men comprised the majority of the workforce such as heavy industry and construction.
Initial symptoms of pleural mesothelioma typically include chest pain and shortness of breath, though you may experience no symptoms at all in the first one or two stages of the cancer’s progression.
The life expectancy of someone with pleural mesothelioma is often less than 18 months, but it depends on many factors, and some patients live much longer with treatment. Combining several treatments, such as surgery, chemotherapy and radiation therapy, has helped people live for years after being diagnosed with Pleural Mesothelioma (PM).
After a person inhales asbestos dust, 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 and chronic inflammation.
The trapped fibers cause pleural mesothelioma by triggering genetic changes that turn cells cancerous. These cancerous cells grow fast and uncontrollably, threatening the organs around them.
Two layers make up the pleura lining. 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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Symptoms of pleural mesothelioma include shortness of breath (dyspnea), chest pain, persistent dry or raspy cough, coughing up blood (hemoptysis) and difficulty swallowing (dysphagia). For many people, symptoms are not noticeable until the cancer is in a later stage.
Three out of four pleural mesothelioma patients report shortness of breath in their first meeting with a doctor, and more than half report chest pain. 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 or nerve pain.
Asbestos fibers can also cause excess fluid to build between the two layers of the pleura, a condition called pleural effusion, which is present in most of the pleural mesothelioma cases. While having a little fluid between the layers is healthy, too much puts pressure on the lungs, causing chest pain that worsens when you cough or take deep breaths.
Doctors sometimes mistake pleural mesothelioma for common diseases with similar symptoms, such as the flu or pneumonia, and the rare cancer requires extensive testing to diagnose.
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 pleural mesothelioma and lung cancer in some cases. While doctors may suspect mesothelioma based on a patient’s scans, symptoms and history of asbestos exposure, 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 examines the biopsy under a microscope to confirm the diagnosis and distinguish the cancer’s cell type.
After a review of their 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.
CT scans provide more advanced images that often show evidence of asbestos exposure.
PET scans can detect signs of cancerous spread to the lymph nodes.
A thoracentesis involves collecting fluid from a pleural effusion through a hollow needle. Pathologists can examine fluid samples for cancer cells, according to the National Comprehensive Cancer Network Guidelines.
After imaging scans, further tests are needed to diagnose mesothelioma with a high level of confidence. In addition to differentiating mesothelioma from lung cancer, determining the mesothelioma stage and cell type is also vital to creating an effective treatment plan.
When no pleural effusion exists, doctors perform biopsies to collect pleural tissue samples. A thoracoscopic biopsy produces the most accurate diagnosis.
If the series of tests and biopsies confirms the presence of mesothelioma, doctors will develop a treatment plan based on the results.Learn more about mesothelioma diagnosis
Several doctors across the nation specialize in the treatment of asbestos-related cancer.
Dr. 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.Contact Dr. Sugarbaker
Dr. Lebenthal specializes in treating lung cancer and mesothelioma at Brigham and Women’s Hospital and the VA Boston Healthcare System, where he works with U.S. veterans diagnosed with the disease.Contact Dr. Lebenthal
Dr. Cameron has helped hundreds of pleural mesothelioma patients at the UCLA Jonsson Comprehensive Cancer Center, and he also plays a crucial role in treating veterans at the VA Greater Los Angeles Healthcare System.Contact Dr. Cameron
A few different staging systems exist for malignant pleural mesothelioma, and they all define the four stages of progressive development. Stages 1 and 2 indicate localized tumors, while stages 3 and 4 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, which is also used to stage many other types of cancer.
Other staging systems used less often include the Brigham system and the Butchart system. Pleural mesothelioma specialist Dr. David Sugarbaker created the Brigham staging system based on his extensive surgical experience. The Butchart system, developed by Dr. Eric Butchart in 1976, is the oldest staging system for pleural mesothelioma.Learn more about pleural mesothelioma staging
Researchers are testing several emerging treatment technologies in their quest for a cure, but mesothelioma specialists still primarily rely on traditional cancer treatments such as surgery, chemotherapy and radiation therapy. Most treatment plans incorporate a combination of the three called multimodal therapy.
Depending on how far your cancer has progressed, these therapies can be cytoreductive or palliative.
Cytoreductive treatments aim to fight the cancer, while palliative treatments address mesothelioma 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. Researchers are constantly experimenting with surgery, chemotherapy and radiation therapy to uncover the most effective combination and timing for these treatments.
It takes a multidisciplinary team to provide multimodal pleural mesothelioma treatment. In one example of a multimodal PM treatment, surgeons first perform extrapleural pneumonectomy surgery, then radiologists administer radiation therapy to reduce the risk of local cancer recurrence, while traditional oncologists prevent distant cancer spread with chemotherapy.Learn more about multimodal treatment
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 and decrease the size of tumors. While recent advancements have improved 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, vinorelbine and doxorubicin.Learn more about chemotherapy
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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
Emerging technologies such as immunotherapy and gene therapy focus on cancer cells directly without harming healthy cells. This targeted approach has the potential to improve outcomes while causing fewer side effects. You may be able to receive an experimental therapy by enrolling in a clinical trial or seeing a mesothelioma specialist.Learn more about clinical trials
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 can ease cancer symptoms and reduce side effects of treatment.
Different types of complementary therapies promote physical, mental and emotional wellness.
Body-based complementary therapies support the health of bones, joints, muscles and the lymphatic and circulatory systems. For example, acupuncture and acupressure are clinically proven to alleviate 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. Yoga breathing techniques sooth the lungs and relieve tension, while meditation reduces anxiety and depression and helps people cope with uncertainty.
Throughout the diagnostic process, doctors analyze the expected course and outcome for the disease — known as your prognosis. Providing an accurate prognosis is challenging because the disease is 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 a 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 also 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 median survival times, 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, and 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.
Cancerous pleural effusions are associated with shorter survival, but treatment with surgery or chemotherapy may extend survival for these patients.
Karen Selby joined Asbestos.com in 2009. She is a registered nurse with a background in oncology and thoracic surgery and was the director of a tissue bank before becoming a Patient Advocate at The Mesothelioma Center. Karen has assisted surgeons with thoracic surgeries such as lung resections, lung transplants, pneumonectomies, pleurectomies and wedge resections. She is also a member of the Academy of Oncology Nurse & Patient Navigators.
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