Mesothelioma

Malignant mesothelioma (me-zo-thee-le-O-muh) is a rare cancer that occurs in the thin layer of tissue that covers the majority of your internal organs (mesothelium).

Doctors divide mesothelioma into different types based on what part of the mesothelium is affected, including:

• Pleural malignant mesothelioma, which affects the tissue that surrounds the lungs and is the most common form of mesothelioma.
• Peritoneal mesothelioma, which occurs in the tissue in your abdomen.
• Pericardial mesothelioma, which affects the tissue surrounding the heart.
• Mesothelioma of the tunica vaginalis, which occurs in the lining around the testicles.

Between 2,000 and 3,000 people are diagnosed with mesothelioma in the United States each year, according to the American Cancer Society.

Symptoms

Signs and symptoms of mesothelioma vary depending on where the cancer occurs.

Pleural mesothelioma signs and symptoms may include:

• Shortness of breath
• Painful breathing (pleurisy)
• Painful coughing
• Chest pain under the rib cage
• Unusual lumps of tissue under the skin on your chest
• Unexplained weight loss
• Dry (nonproductive) cough

Peritoneal mesothelioma signs and symptoms may include:

• Abdominal pain
• Abdominal swelling
• A change in your bowel habits, such as more frequent diarrhea or constipation
• Lumps of tissue in the abdomen
• Unexplained weight loss

Other forms of mesothelioma
Signs and symptoms of pericardial mesothelioma and mesothelioma of the tunica vaginalis are unclear. These forms are so rare that not much information is available. Pericardial mesothelioma signs and symptoms may include difficulty breathing or chest pains. Mesothelioma of the tunica vaginalis may be first detected as a mass on a testicle.

When to see a doctor
See your doctor if you have signs and symptoms that may indicate mesothelioma. Signs and symptoms of mesothelioma aren't specific to this disease and, due to the rarity of mesothelioma, are more likely to be related to other conditions. If any persistent signs and symptoms seem unusual or bothersome to you, ask your doctor to evaluate them.

Causes

In general, cancer begins when a series of genetic mutations occur within a cell, causing the cell to grow and multiply out of control, when healthy cells would normally die. The accumulating abnormal cells form a mass (tumor). Cancer cells invade nearby tissues and can break off from an initial tumor to spread elsewhere in the body (metastasize).

It isn't clear what causes the initial genetic mutations that lead to mesothelioma, though researchers have identified factors that may increase the risk. It's likely that cancers form because of an interaction between many factors, such as inherited conditions, your environment, your health conditions and your lifestyle choices.

Benign pleural mesothelioma
A form of noncancerous (benign) tumor that can occur in the chest is sometimes called benign mesothelioma. However, this name is misleading. Benign mesothelioma doesn't begin in the same cells where the cancerous forms of mesothelioma begin. And, in a minority of cases, benign mesothelioma can be very aggressive, despite the term "benign." For this reason, some doctors now refer this tumor as solitary fibrous tumor.

Solitary fibrous tumor usually doesn't cause signs and symptoms. Most cases are inadvertently discovered during tests and procedures for other conditions. It isn't clear what causes solitary fibrous tumors, but they aren't linked to asbestos exposure. Treatment for solitary fibrous tumor typically includes surgery.

Risk factors

Asbestos exposure: The primary risk factor for mesothelioma
Asbestos is a mineral that's found naturally in the environment. Asbestos fibers are strong and resistant to heat, making them useful in a wide variety of applications, such as in insulation, cement, brakes, shingles, flooring and many other products. People who work around asbestos fibers are thought to have the greatest risk of mesothelioma.

When asbestos is broken up, such as during the mining process or when removing asbestos insulation, dust may be created. If the dust is inhaled or swallowed, the asbestos fibers may settle in the lungs or in the stomach, where they can cause irritation that may lead to mesothelioma, though how exactly this happens isn't understood.

Mesothelioma risk is believed to be increased in people who are exposed to high levels of asbestos, in people who are exposed to asbestos over a long period of time and in people exposed to asbestos at a young age. It can take 30 to 40 years or more for mesothelioma to develop as a result of asbestos exposure.

Some people with years of asbestos exposure never develop mesothelioma. And yet, others with very brief exposure develop the disease. This indicates that other factors may be involved in determining whether someone gets mesothelioma or doesn't. For instance, you could inherit a predisposition to cancer or some other condition could increase your risk.

Possible risk factors
Factors that may increase the risk of mesothelioma include:

• Personal history of asbestos exposure. If you've been directly exposed to asbestos fibers at work or at home, your risk of mesothelioma is greatly increased.
• Living with someone who works with asbestos. People who are exposed to asbestos may carry the fibers home on their skin and clothing. These stray fibers can put others in the home at risk of mesothelioma. People who work with asbestos should shower and change clothes before leaving work.
• Smoking. Risk of mesothelioma is increased greatly in smokers who are exposed to asbestos.
• SV40. Some research indicates a link between mesothelioma and simian virus 40 (SV40), a virus originally found in monkeys. Millions of people may have been exposed to SV40 when receiving polio vaccinations between 1955 and 1963, because the vaccine was developed using monkey cells. Once it was discovered that SV40 was linked to certain cancers, the virus was removed from the polio vaccine. Whether SV40 increases the risk of mesothelioma is a point of debate, and more research is needed.
• Radiation. Some research links mesothelioma to the radioactive substance thorium dioxide, which was used along with X-rays to diagnose various health conditions from the 1920s to the 1950s. Thorium dioxide was later found to cause cancer and is no longer used.
• Family history. A family history of mesothelioma may increase your risk of mesothelioma, but more research is needed to understand this theory.

Complications

Complications from spreading cancer
As pleural mesothelioma spreads in the chest, it puts pressure on the structures in that area. This can cause complications, such as:

• Difficulty breathing
• Chest pain
• Difficulty swallowing
• Swelling caused by pressure on the large vein that leads from your upper body to your heart (superior vena cava syndrome)
• Pain caused by pressure on the nerves and spinal cord
• Accumulation of fluid in the chest (pleural effusion), which can compress the lung nearby and make breathing difficult

Death
Mesothelioma that progresses can lead to death. People who die of mesothelioma usually die from related complications, such as lung failure, heart problems, stroke and other causes.

Preparing for your appointment

You're likely to start by first seeing your family doctor or a general practitioner. However, in some cases when you call to set up an appointment, you may be referred immediately to a doctor who specializes in lung diseases (pulmonologist) or abdominal problems (gastroenterologist).

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well prepared for your appointment. Here's some information to help you get ready for your appointment, and what to expect from your doctor.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes. If you're worried about mesothelioma, make a list of all your occupations, even those you had for only a few months.
• Make a list of all medications, as well as any vitamins or supplements, that you're taking.
• Take a family member or friend along, if possible. Sometimes it can be difficult to soak up all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions will help you make the most of your time together. List your questions from most important to least important in case time runs out. For mesothelioma, some basic questions to ask your doctor include:

• What is likely causing my symptoms or condition?
• What are other possible causes for my symptoms or condition?
• What kinds of tests do I need?
• Is my condition likely temporary or chronic?
• What is the best course of action?
• What are the alternatives to the primary approach that you're suggesting?
• I have these other health conditions. How can I best manage them together?
• Are there any restrictions that I need to follow?
• Should I see a specialist? What will it cost, and will my insurance cover seeing a specialist?
• Is there a generic alternative to the medicine you're prescribing me?
• Are there any brochures or other printed material that I can take home with me? What Web sites do you recommend?
• What will determine whether I should plan for a follow-up visit?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.

What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may allow more time to cover other points you want to address. Your doctor may ask:

• When did you first begin experiencing symptoms?
• Have your symptoms been continuous, or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?
• Do your symptoms affect your ability to work?

What you can do in the meantime
Try to avoid anything that worsens your signs and symptoms. For instance, if you're experiencing shortness of breath, try to take it easy until you can meet with your doctor.

Tests and diagnosis

If you have signs and symptoms that might indicate mesothelioma, your doctor will conduct a physical exam to check for any lumps or other unusual signs. Your doctor may order imaging scans, such as a chest X-ray or a computerized tomography (CT) scan of your chest or abdomen, to look for abnormalities.

It's not uncommon for mesothelioma to be misdiagnosed initially because mesothelioma is rare, and its signs and symptoms aren't specific. Your doctor will likely rule out other more common conditions before considering mesothelioma.

Biopsy
Biopsy, a procedure to remove a small portion of tissue for laboratory examination, is the only way to determine whether you have mesothelioma. Depending on what area of your body is affected, your doctor selects the right biopsy procedure for you. Options include:

• Fine-needle aspiration. The doctor removes fluid or a piece of tissue with a small needle inserted into your chest or abdomen.
• Thoracoscopy. Thoracoscopy allows the surgeon to see inside your chest. In this procedure, the surgeon makes one or more small incisions between your ribs. A tube with a tiny video camera is then inserted into your chest cavity — a procedure sometimes called video-assisted thoracoscopic surgery (VATS). Special surgical tools allow your surgeon to cut away tissue for testing.
• Laparoscopy. Laparoscopy allows the surgeon to see inside your abdomen. Using one or more small incisions into your abdomen, the surgeon inserts a tiny camera and special surgical tools to obtain a small piece of tissue for examination.
• Thoracotomy. Thoracotomy is surgery to open your chest between the ribs to allow a surgeon to check for signs of disease. He or she removes a sample of tissue for testing.
• Laparotomy. Laparotomy is surgery to open your abdomen to allow a surgeon to check for signs of disease. He or she removes a sample of tissue for testing.

The tissue sample is analyzed under a microscope to see whether the abnormal tissue is mesothelioma and what types of cells are involved. The type of mesothelioma you have determines your treatment plan.

Staging
Once mesothelioma is diagnosed, your doctor orders other tests to determine the extent, or stage, of the cancer. Imaging tests that may help determine the stage of your cancer include:

• Chest X-ray
• CT scans of the chest and abdomen
• Magnetic resonance imaging (MRI)
• Positron emission tomography (PET)

Once the extent of pleural mesothelioma is determined, a stage is assigned. Formal stages aren't available for other types of mesothelioma because these types are rare and aren't well studied. The stages of pleural mesothelioma are:

• I. Stage I pleural mesothelioma is considered localized cancer, meaning it's limited to one portion of the lining of the chest.
• II. Stage II mesothelioma may have spread beyond the lining of the chest to the diaphragm or to a lung.
• III. Stage III mesothelioma may have spread to other structures within the chest and may involve nearby lymph nodes.
• IV. Stage IV mesothelioma is an advanced cancer that has spread to distant areas (metastasized). Mesothelioma most commonly spreads (metastasizes) to the brain, lymph nodes in the chest and areas of the lung that are away from the tumor.

Treatments and drugs

What treatment you undergo for mesothelioma depends on your health and certain aspects of your cancer, such as its stage and location. Unfortunately, mesothelioma often is an aggressive disease and for most people a cure isn't possible. Mesothelioma is usually diagnosed at an advanced stage — when it isn't possible to remove the cancer through an operation. Instead, your doctor may work to control your cancer to make you more comfortable.

Discuss treatment goals with your doctor. Some people want to do everything they can to treat their cancer, even if that means enduring side effects for a small chance of an improvement. Others prefer treatments that make them comfortable so that they can live their remaining months as symptom-free as possible.

Surgery
Surgeons work to remove mesothelioma in instances where it is diagnosed at an early stage. Sometimes it isn't possible to remove all of the cancer. In those cases, surgery may help to reduce the signs and symptoms caused by mesothelioma spreading in your body. Surgical options may include:

• Surgery to decrease fluid buildup. Pleural mesothelioma may cause fluid to build up in your chest, causing difficulty breathing. Surgeons insert a tube or catheter into your chest to drain the fluid. Surgeons may also inject medicine into your chest to prevent fluid from returning (pleurodesis).
• Surgery to remove the tissue around the lung or abdomen. Surgeons may remove the tissue lining the ribs and the lungs (pleurectomy) or the tissue lining the abdominal cavity (peritonectomy) in order to relieve signs and symptoms of mesothelioma.
• Surgery to remove as much of the cancer as possible (debulking). If all of the cancer can't be removed, surgeons may attempt to remove as much as possible.
• Surgery to remove a lung and the surrounding tissue. Removing the affected lung and the tissue that surrounds it may relieve signs and symptoms of pleural mesothelioma. This procedure also allows doctors to use higher doses of radiation against any remaining mesothelioma, since they won't need to worry about protecting your lung from damaging radiation.

Chemotherapy

Chemotherapy uses chemicals to kill cancer cells. Systemic chemotherapy travels throughout the body and may shrink or slow the growth of a pleural mesothelioma that can't be removed using surgery. Chemotherapy may also be used before surgery (neoadjuvant chemotherapy) to make an operation easier or after surgery (adjuvant chemotherapy) to reduce the chance that cancer will return.

Chemotherapy drugs may also be heated and administered directly into the abdominal cavity (intraperitoneal chemotherapy), in the case of peritoneal mesothelioma, or into the chest cavity (intrapleural chemotherapy), in the case of pleural mesothelioma. Using this strategy, chemotherapy drugs can reach the mesothelioma directly without injuring healthy cells in other parts of the body. This allows doctors to administer higher doses of chemotherapy drugs.

Intraperitoneal chemotherapy may also be used to reduce the signs and symptoms of peritoneal mesothelioma that can't be removed through surgery.

Radiation therapy

Radiation therapy focuses high-energy beams to a specific spot or spots on your body. Radiation may reduce signs and symptoms in people with pleural mesothelioma. Doctors aim radiation at the entire chest to obtain the best result. However, many sensitive organs are in the chest, such as the heart, lungs, esophagus and spinal cord, so doctors must use low doses of radiation to spare these organs. Radiation therapy is sometimes used after biopsy or surgery to prevent mesothelioma from spreading to the surgical incision.

Radiation therapy is used occasionally in people with peritoneal mesothelioma to reduce signs and symptoms caused by the cancer.

Combination therapy

Surgery, chemotherapy and radiation therapy may be combined. This aggressive therapy can be grueling and may not be appropriate for everyone. Younger, healthier people and those with earlier stage mesothelioma may be more able to endure this treatment. Combination therapy has shown the most promise in treating mesothelioma. However, most people will eventually experience a recurrence of this cancer despite aggressive treatment. Combination therapy has been used in both pleural mesothelioma and peritoneal mesothelioma.

Clinical trials

Clinical trials are studies of new mesothelioma treatment methods. People with mesothelioma may opt for a clinical trial for a chance to try new types of treatment. However, a cure isn't guaranteed. Carefully consider your treatment options and talk to your doctor about what clinical trials are open to you. Your participation in a clinical trial may help doctors better understand how to treat mesothelioma in the future.

Clinical trials are currently investigating a number of targeted drugs. Targeted drug therapy uses drugs to attack specific abnormalities within cancer cells. Targets being studied in mesothelioma include a substance that cancer cells make to attract new blood vessels to bring the cancer oxygen and nutrients. Another target is an enzyme that helps cancer cells develop resistance to chemotherapy drugs. Researchers hope drugs that target these areas can help kill mesothelioma cells.

Treatment for other types of mesothelioma

Pericardial mesothelioma and mesothelioma of the tunica vaginalis are very rare and can be very aggressive. Early-stage cancer may be removed through surgery. Doctors have yet to determine the best way to treat later stage cancers, though. Your doctor may recommend other treatments to improve your quality of life.

Alternative medicine

No alternative medicine treatments have proved helpful in treating mesothelioma. But complementary and alternative mesothelioma treatments may help control signs and symptoms. Discuss options with your doctor.

Mesothelioma can cause pressure within your chest that can make you feel as though you're always short of breath. Breathlessness can be distressing. Your doctor may recommend using an oxygen mask or taking medications to make you more comfortable, but often these aren't enough. Combining your doctor's recommended treatments with complementary and alternative approaches may help you feel better.

Alternative treatments that have shown some promise in helping people cope with breathlessness include:

• Acupuncture. Acupuncture uses thin needles inserted at precise points into your skin.
• Breath training. A nurse or physical therapist can teach you breathing techniques to use when you feel breathless. Sometimes you may feel breathless and begin to panic. Implementing these techniques may help you feel more in control of your breathing.
• Relaxation exercises. Slowly tensing and relaxing different muscle groups may help you feel more at ease and breathe easier. Your doctor may refer you to a therapist who can teach you relaxation exercises so that you can do them on your own.
• Sitting near a fan. Directing a fan to your face may help ease the sensation of breathlessness.

Coping and support

A diagnosis of mesothelioma can be devastating not only to you, but also to your family and friends. Take time to experience the sadness and despair and to grieve. And remember that you decide how you'll spend your time and with whom you'll spend it.

In order to regain a sense of control, try to:

• Learn everything you can about mesothelioma. Write down a list of questions to ask your doctor. Ask your health care team for information to help you better understand your disease. Consult the National Cancer Institute (NCI) and the American Cancer Society (ACS). Both organizations have Web sites and toll-free telephone information lines. Call the NCI at 800-4-CANCER (800-422-6237). Call the ACS at 800-ACS-2345 (800-227-2345).
• Surround yourself with a support network. Close friends or family can help you with everyday tasks, such as getting you to appointments or treatment. If you have trouble asking for help, learn to be honest with yourself and accept help when you need it.
• Seek out other people with cancer. Ask your health care team about cancer support groups in your community. Sometimes there are questions that can only be answered by other people with cancer. Support groups offer a chance to ask these questions and receive support from people who understand your situation. Online support message boards, such as the ACS's Cancer Survivors Network, can offer similar benefits while allowing you to remain anonymous.
• Plan for the unknown. Ask your health care team about advance directives that give your family guidance on your medical wishes in case you can no longer speak for yourself. Talk to a lawyer about your will, if you haven't already done so.

Prevention

Reducing your exposure to asbestos may lower your risk of mesothelioma.

Find out whether you work with asbestos
Most people with mesothelioma were exposed to the asbestos fibers at work. Workers who may encounter asbestos fibers include:

• Miners
• Factory workers
• Insulation manufacturers
• Railroad workers
• Ship builders
• Gas mask manufacturers
• Construction workers
• Auto mechanics

Ask your employer whether you have a risk of asbestos exposure on the job.

Follow your employer's safety regulations
Follow all safety precautions in your workplace, such as wearing protective equipment. You may also be required to shower and change out of your work clothes before taking a lunch break or going home. Talk to your doctor about other precautions you can take to protect yourself from asbestos exposure.

Be safe around asbestos in your home

Older homes and buildings may contain asbestos. In many cases, it's more dangerous to remove the asbestos than it is to leave it intact. Breaking up asbestos may cause fibers to become airborne, where they can be inhaled. Consult experts trained to detect asbestos in your home. These experts may test the air in your home to determine whether the asbestos is a risk to your health. Don't attempt to remove asbestos from your home — hire a qualified expert. The Environmental Protection Agency offers advice on its Web site for dealing with asbestos in the home.

Source:www.mayoclinic.com