Vogelzang Law Guide to Mesothelioma
(Updated July 2017)

Progress in determining the cause of mesothelioma was slow and during the early years, there were many disagreements. Read this Guide to Mesothelioma for information about the cancer known as mesothelioma.

History of Mesothelioma

The history of mesothelioma is a winding path through many years of uncertainty and disagreements. (This information was condensed from the monograph, A History of Mesothelioma, by pulmonary specialist Dr. Dorsett D. Smith.)

A Brief Look at the Long History of Mesothelioma

Since mesothelioma cancer is rare, there were few cases to study. Even when researchers did conduct studies, there was not enough data to confirm the progression of the disease, the cause of the disease or even if mesothelioma could be considered its own disease.

Slowly, over the decades, researchers made progress. Here are a few highlights from the early years of mesothelioma history:

  • 1767 - Frenchman Joseph Lieutaud is credited with the earliest mention of a possible tumor in the chest wall. In his published study of 3,000 autopsies, Lieutaud mentioned two cases of "pleural tumors".
  • 1843 - Austrian pathologist Karl, Baron von Rokitansky was the first to offer a pathologic description of the peritoneal mesothelioma
  • 1890 - Biggs identified the first American mesothelioma case
  • 1920 - Ernest S. Du Bray first coined the term “mesothelioma”
  • 1933 - Researcher S. Roodhouse Gloyne came very close to associating the cause of mesothelioma with asbestos; he ended up rejecting the idea.
  • 1933 - German researcher H. W. Wedler first reported an unusual form of pleural malignancy in 30 autopsies on asbestos workers. He excluded one case, and of the 29 remaining autopsies, four had bronchial cancers, and two others had a malignant pleural growth. Wedler's research was accepted in Germany. Unfortunately, other researchers around the globe were suspicious, due to the political climate created with the rise of Hitler.

Mesothelioma Research Accelerates

During the 1960s, research reports came in quick succession that began pointing to asbestos as the cause of this cancer. Here is a brief timeline:

  • 1960 – South African researcher J.C. Wagner associated mesothelioma with northwest Cape crocidolite, a form of asbestos
  • 1960 – In reviewing the records of an English hospital, E.E. Keel discovered four women diagnosed with carcinomatosis of the peritoneum without a known primary; one woman diagnosed with ovarian cancer, and four other women diagnosed with peritoneal malignancy, possibly of ovarian origin. Keel suspected an association with asbestosis and peritoneal cancer, but the connection was not strongly suggested until four years later.
  • 1963 – R.R. Tomson reported asbestos bodies in the lungs of South Africans who were not asbestos workers and called it a modern urban hazard.
  • 1964 – John Enticknap discerned an association of asbestos with peritoneal mesothelioma
  • 1965 – American researcher Irving J. Selikoff presents a paper at the New York Academy of Science Symposium; his thesis was the association between asbestos and mesothelioma.
  • 1968 – H.M. Utidjian et al, reported that almost 100 percent of urban dwellers had asbestos bodies in their lungs

Concurrent with the advancing research on mesothelioma, British society began changing as well. By 1966 the importation of crocidolite asbestos had been voluntarily abandoned in England, and new asbestos regulations accepting the relationship between asbestos and mesothelioma were adopted in 1969.

Moving Towards Agreement About the Causes of Mesothelioma

The 1970s was the decade when the association between asbestos exposure and malignant mesothelioma was generally accepted. Here are a few highlights from that decade:

·         1970 - Thompson’s original observations were widely confirmed in: Montreal, Milan, London, Newcastle, Glasgow, Belfast, Dresden, Pittsburgh, Miami, New York

·         1973 – Now both the criteria for diagnosis and the clear association between asbestos exposure and mesothelioma are generally accepted

In the decades that followed, research delved more deeply into the relationship between asbestos exposure and mesothelioma cancer, such as determining the size and shape of asbestos fibers caused malignant mesothelioma.


 

 

Mesothelioma Recognized as a Cancer

Cancer of the mesothelium (the thin layer of tissue covering the abdomen, lung or chest wall and sometimes in the heart or testicles) is called malignant mesothelioma. The NIH estimates that 90 percent of mesothelioma cancers are caused by exposure to asbestos, 20 to 40 years previous to the diagnosis.

Mesothelioma is further characterized by what cells in the mesothelium it affects. The characterizations include:

  • Epithelial- The epithelium is the first layer or layers of the mesothelium. It is aptly called "epithelial mesothelioma. This form grows more slowly than other forms of malignant mesothelioma and is generally the easiest to treat. 
  • Sarcomatoid - These cells are spindle shaped in the mesothelium
  • Mixed - When mesothelioma cancer is present in both epithelial and sarcomatoid cells

This cancer shows up in people who were directly exposed to asbestos in the workplace and sometimes in their family members as well.

Types of malignant mesothelioma

The types of mesothelioma cancer include:

  • Pleural mesothelioma – cancer in the lining of your lungs or chest cavity
  • Peritoneal mesothelioma – cancer in the lining of your abdomen
  • Pericardial mesothelioma – cancer in the lining of your heart
  • Testicular mesothelioma – cancer in the lining of the testes

Pericardial and testicular mesothelioma are rarer forms of the cancer than are pleural and peritoneal mesothelioma.

Treatment options for mesothelioma cancer

The four common modalities to treat malignant mesothelioma are:

  • Surgery – This modality includes two different techniques: 
    • Using a scalpel to cut out the tumor and surrounding tissue
    •  Injecting chemicals to create scar tissue to prevent fluid build-up in the affected area
  •  Radiation therapy – Treating the malignancy with x-rays and other forms of radiation
  • Chemotherapy – A treatment that uses combinations of chemicals known to shrink the malignancy
  • Targeted therapy – The use of drugs or other substances to “target” specific areas of the cancer

In addition to these top four treatment options, other therapies include:  photodynamic therapy, virus, gene and immunotherapy.

Mesothelioma cancer prognosis

There are many factors and combinations of factors that affect your mesothelioma cancer prognosis. Some of them include: 

  • The type of malignant mesothelioma – This includes where it is located in the body
  • The stage of the mesothelioma cancer – A “stage” refers to the size of the cancer and where it is located in your body
  • The grade of the mesothelioma cancer - A “grade” refers to how abnormal the cancer cells look under a microscope. Grading provides clues about how quickly the cancer is likely to grow and spread.
  • Certain traits of the mesothelioma cancer cells
  • Your age and how healthy you were before your malignant mesothelioma cancer – Generally speaking, the younger and healthier you are, the better your prognosis
  • How you respond to treatment

Only an experienced mesothelioma cancer physician can recommend the best treatment for the best possible malignant mesothelioma prognosis.

Mesothelioma symptoms 

Symptoms of pleural mesothelioma

Mesothelioma affecting the tissue surrounding the lungs (pleural mesothelioma) can cause signs and symptoms that can include:

  • Chest pain under the rib cage
  • Painful coughing
  • Prolonged coughing
  • Shortness of breath
  • Unusual lumps of tissue under the skin on your chest
  • Unexplained weight loss
  • Fatigue
  • Collapse of lung
  • Blood in sputum (phlegm) coughed up from the lungs
  • Difficulty swallowing
  • Prolonged hoarseness
  • Nausea
  • Low oxygen levels

Mesothelioma found in the abdomen (peritoneal mesothelioma) can cause the following signs and symptoms:

  • Abdominal pain
  • Abdominal swelling
  • Lumps of tissue in the abdomen
  • Unexplained weight loss
  • Fatigue

Other forms of mesothelioma

Signs and symptoms of other types of mesothelioma are unclear, since these forms of the disease are very rare.

  •  Pericardial mesothelioma (mesothelioma found in the membrane surrounding the heart) can cause symptoms like: 
    • Breathing difficulty
    •  Chest pains
  • Mesothelioma of tunica vaginalis (mesothelioma in the tissue surrounding the testicles) can cause symptoms such as a swelling or a mass on a testicle

When to see a doctor

Considered a rare cancer, malignant mesothelioma can be difficult to diagnose. Not just because of its rarity, but also because the symptoms you experience could be due to another type of cancer, such as lung cancer. That’s why it’s important to see an experienced mesothelioma physician to ensure your diagnosis is accurate.

That said, regardless whether it’s lung cancer or mesothelioma, it’s important you do see a physician, if you are experiencing one or more of the symptoms described above and you’ve been exposed to asbestos 20 or more years ago.

Ways your doctor can use to diagnose mesothelioma

Some techniques your doctor may use to diagnose your mesothelioma include: 

Physical tests

  • Needle biopsy
  • Drainage of lung fluid
  • Lung function test

Imaging tests, which may include:

  • CT or CAT (computed axial tomography) scans
  • MRI (magnetic resonance imaging) scans
  • PET (positron emission tomography) scans
  • Chest X-ray

Thoracoscopic surgical biopsy: A small incision (cut) is made in the chest. A tiny tube with a camera on the end is inserted, and a small amount of tissue is removed and looked at under a microscope.

Types of Mesothelioma

Compared to many other cancers, the prognosis for mesothelioma is not a good one. According to the most recent statistics provided by the American Cancer Society, the relative five-year survival rate is between 5 to 10 percent. Even when the cancer is caught at its earliest stage, most people with a mesothelioma prognosis have less than two years to live.

mesothelioma

Mesothelioma is a cancer of the lining of your organs. This cancer can appear in one of three areas of the body: 

  • Pleural mesothelioma – cancer in the lining of your lungs
  • Peritoneal mesothelioma – cancer in the lining of your abdomen
  • Pericardial mesothelioma – cancer in the lining of your heart
  • Metastatic mesothelioma– cancer that has spread beyond its origin
  • Epitheliod mesothelioma– the broadest category for malignant mesothelioma

What is pleural mesothelioma?

The phrase, pleural mesothelioma, describes the location of your malignant mesothelioma. In this case, it is the pleural cavity, your lungs.

What are the symptoms of pleural mesothelioma?

As this mesothelioma cancer resides in your lungs, your symptoms will be related to your thoracic cavity (chest), pleural cavity (lungs) and your breathing. They include:

  • Having trouble breathing
  • Persistent coughing that gets worse
  • Experiencing a pain under the rib cage
  • Experiencing a weight loss for no known reason
  • Constantly feeling tired

If you are experiencing any of these symptoms, it is wise to contact a physician experienced in mesothelioma.

How is pleural mesothelioma diagnosed?

Sometimes it is hard to tell the difference between malignant mesothelioma in the chest and lung cancer. However, by using the following tests and procedures, your physician will be able to determine which cancer it is.

  • Physical exam and history - An exam of the body to check general signs of health, including checking for signs of disease and a history of the patient’s health habits, exposure to asbestos, and past illnesses and treatments will also be taken.
  • Chest x-ray - An x-ray of the organs and bones inside the chest.
  • CT scan (CAT scan) - A procedure that makes a series of detailed, computer-generated pictures of the chest, taken from different angles. Also called: computed tomography, computerized tomography, or computerized axial tomography.
  • Biopsy - The removal of cells or tissues from the pleura so they can be viewed under a microscope by a pathologist to check for signs of cancer.

Only an experienced pleural mesothelioma physician can determine what test is right for you.

Testing for pleural mesothelioma on the cellular level

Procedures used to collect the cells or tissues include the following:

  • Fine-needle aspiration (FNA) biopsy of the lung - The removal of tissue or fluid from the lung using a thin needle.
  • Thoracoscopy  - An incision (cut) is made between two ribs and a thoracoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted into the chest.
  • Thoracotomy - An incision (cut) is made between two ribs to check inside the chest for signs of disease.
  • Open biopsy - A procedure in which an incision (cut) is made through the skin to expose and remove tissues to check for signs of disease.

By examining cells or tissues, a pathologist can help determine if you have pleural mesothelioma.

Here are some of the things you can expect when your doctor suspects you may have peritoneal mesothelioma.

What is “peritoneal mesothelioma”?

Peritoneal mesothelioma is a mesothelioma cancer located in the peritoneum, the lining of the abdomen.

What are the symptoms of peritoneal mesothelioma?

  • Pain or swelling in the abdomen
  • Lumps in the abdomen
  • Constipation
  • Weight loss for no known reason
  • Feeling very tired.

If you are experiencing any of these symptoms, it is wise to see your doctor.

How is peritoneal mesothelioma diagnosed?

There are various tests and procedures to diagnose peritoneal mesothelioma. They include:

  • Physical exam and history - An exam of the body to check general signs of health, including checking for signs of disease and a history of the patient’s health habits, exposure to asbestos, and past illnesses and treatments will also be taken.
  • CT scan (CAT scan) - A procedure that makes a series of detailed, computer-generated pictures of the abdomen, taken from different angles. Also called: computed tomography, computerized tomography, or computerized axial tomography.
  • Biopsy - The removal of cells or tissues from the peritoneum so they can be viewed under a microscope by a pathologist to check for signs of cancer.

Only an experienced peritoneal mesothelioma physician can determine what test is right for you.

ASSESSING peritoneal mesothelioma on the cellular level

Procedures used to collect the cells or tissues include the following:

  • Fine-needle aspiration (FNA) biopsy of the lung - The removal of tissue or fluid using a thin needle. An imaging procedure is used to locate the abnormal tissue or fluid in the abdomen. A small incision may be made in the skin where the biopsy needle is inserted into the abnormal tissue or fluid, and a sample is removed.
  • Peritoneoscopy - An incision (cut) is made in the abdominal wall and a peritoneoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted into the abdomen.
  • Laparotomy - An incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease.
  • Open biopsy - A procedure in which an incision (cut) is made through the skin to expose and remove tissues to check for signs of disease.

By examining cells or tissues, a pathologist can help determine if you have peritoneal mesothelioma.

Epithelioid Mesothelioma

According to the National Cancer Institute, epithelioid mesothelioma is the most common form of malignant mesothelioma and one of the easiest to cure. Generally speaking, treatment for epithelioid mesothelioma is multi-modal (using different types of treatment). Your doctor may also offer you the opportunity to take part in a clinical trial.

A cancer, malignant mesothelioma is most usually found in the lining in three areas of the body. They are:  

  • Lung - pleural mesothelioma
  • Chest wall – also known as pleural mesothelioma
  • Abdomen – peritoneal mesothelioma

Epithelioid mesothelioma describes an even more specific location of this cancer. It is found in the outermost layer of those cells (epithelium) lining the lung, chest wall and abdomen. 

The most common cause of epithelioid mesothelioma is exposure to asbestos.

Symptoms of malignant mesothelioma

These symptoms are:

  • Having trouble breathing
  • Persistent coughing
  • Experiencing a pain under the rib cage
  • Experiencing pain or swelling in the abdomen
  • Finding lumps in the abdomen
  • Persistent constipation
  • Having problems with blood clots (clots form when they shouldn’t)
  • Experiencing a weight loss for no known reason
  • Constantly feeling very tired

Although the following symptoms may have other causes, if you are experiencing them – particularly if you’ve been exposed to asbestos – then you should see your doctor.

Diagnosing epithelioid mesothelioma

Although oncologists (cancer doctors) experienced in treating epithelioid mesothelioma know that the most likely cause is asbestos exposure, they also know it can be difficult to properly diagnose. That’s because it doesn’t appear until decades after the asbestos exposure and, in some cases, resembles other types of cancer.

Treatments

Treatments for epithelioid mesothelioma include:

  • Surgery – Physically removing the cancer
  • Radiation therapy – Treating the malignancy with radiologic (radiation-producing) elements
  • Chemotherapy – A treatment that uses combinations of chemicals known to shrink the malignancy
  • Photodynamic therapy – A specific drug is injected in the patient’s veins where it collects within the cancer. Later a light is shone on the cancer, activating the drug to destroy the cancer.
  • Targeted therapy – Treatment that targets specific areas of the cancer, on the cellular level
  • Virus therapy – Using a virus to attack the cancer cells
  • Gene therapy – Attacking the cancer on the genetic level
  • Immunotherapy – Creating a cancer-fighting vaccine that stimulates the body’s own immune system to attack the cancer

Although epithelioid mesothelioma can be treated using two or more treatment options, which treatment options to use depends upon many factors, so that decision is best left to your physician.

Mesothelioma Treatment Options

Common mesothelioma treatment options are many and depend upon the type and extent of the malignant mesothelioma. Added to these options are others that recently have emerged from clinical trials. Only your mesothelioma physician can determine what is best for you.

Mesothelioma Treatments

According to the National Cancer Institute, there are four common treatment options for mesothelioma. They are:

  1. Surgery – This therapy either physically removes the tumor and surrounding tissue or uses chemicals to prevent accumulation of fluid.
  2. Chemotherapy -  Using a chemical or combination of chemicals, this treatment is used to ensure all the cancer cells are killed.
  3. Radiation therapy - Treating the malignancy with high-energy x-rays. This treatment can be either internal or external.
  4. Targeted therapy - Treatment that uses drugs or other substances to “target” specific areas of the cancer, often on the cellular level

Surgery

Surgery for malignant mesothelioma can take many forms. Here are some surgical techniques used to remove cancer in the lungs and chest (pleural) cavity:

  • Wide local excision: Removing the cancer and some of the healthy tissue surrounding the malignancy.
  • Pleurectomy and decortication: In this technique, part of the lung's covering, the lining of the chest and part of the outside surface of the lungs is removed
  • Extrapleural pneumonectomy: Means the surgical removal of the lining of the sac (pericardium) of the heart, part of the lining of the diaphragm and chest and one whole lung
  • Pleurodesis: With this technique, using a catheter or chest tube, the fluid that has accumulated in the pleural cavity is first drained. Then, using that same tube, chemicals or drugs are poured, causing scarring in the space between the layers of the pleura. The purpose behind the scarring is to stop the build-up of fluid in the pleural cavity.

Even if the malignant mesothelioma has been successfully removed by surgery, frequently chemotherapy or radiation therapy is ordered to ensure all the cancer cells are killed.

Chemotherapy

Chemotherapy uses drugs to either kill the cancer cells or stop the cells from growing. The way chemotherapy is administered depends upon the type and stage of the malignant mesothelioma being treated. For example:

  •  Systemic chemotherapy - Can be taken by mouth or injected into a vein or muscle. The chemicals flow through the entire body system.
  • Regional chemotherapy - Sometimes chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the chest or peritoneum. The drugs affect the cancer cells in a particular area (region) of the body.
  • Hyperthermic intraperitoneal chemotherapy - This treatment is used for malignant mesothelioma that has spread to the tissue that lines the abdomen and that covers most of the organs in the abdomen (peritoneum). It uses a heated solution of anticancer drugs pumped into and out of the abdomen.

Radiation therapy

This treatment uses radiant energy (x-rays or other kinds of radiation) to either kill malignant mesothelioma cells outright or to prevent them from growing and spreading. The two main types of radiation therapy are:

  1. External radiation therapy - In this therapy, a machine positioned outside the body is used to send radiation toward the malignant mesothelioma
  2. Internal radiation therapy - In this form of therapy, a radioactive substance is sealed in needles, wires, seeds, or catheters and placed directly near or into the malignancy.

Only your experienced oncologist can determine which course of radiation therapy is best.

Targeted Therapy

Unlike chemotherapy or radiation, targeted therapy is less likely to harm healthy cells. This therapy uses drugs or other substances to “target” malignant mesothelioma cells. Targeted therapy includes:

  • Monoclonal antibody therapy - Using antibodies created in a laboratory from a certain type of immune system cell, this therapy targets the substances on cancer cells that help them grow. These antibodies attach themselves to those substances, killing the cancer cells, blocking their growth or keeping them from spreading.
  • Bevacizumab - A type of monoclonal antibody, Bevacizumab treats advanced malignant mesothelioma. It works by binding with a blood protein (vascular endothelial growth factor, or VEGF), preventing the malignant mesothelioma from growing new blood vessels, in effect, "starving" the tumor.
  • Kinase inhibitors - This targeted therapy works by blocking the growth signals sent out by the malignant mesothelioma tumors.

Targeted therapy for malignant mesothelioma works on the biochemical level to kill or stop cancer cells from growing.

Other malignant mesothelioma treatment options

In addition to the four common treatments, there are other treatments. They are:

  • Photodynamic therapy – A light-activated drug is injected into a patient’s veins. This drug collects within the cancer. When a light is shone on the cancer, the drug is activated, killing the malignant mesothelioma.
  • Virus therapy – Just as the term sounds, this therapy uses a virus to attack the cancer cells
  • Gene therapy – This therapy attacks the malignancy on the genetic level  
  • Immunotherapy – Using a cancer-fighting vaccine to stimulate the body’s own immune system, the malignant mesothelioma is attacked by the body’s own processes

This is not an exhaustive list of treatments, as clinical trials are ongoing with new treatments emerging nearly every day.

Mesothelioma prognosis

When mesothelioma affects key organs like the lungs or heart, generally it is more difficult to treat than if found in another area of the body, such as the abdomen.

Factors affecting your prognosis

Generally speaking, you have the greatest chance for successfully beating mesothelioma:

  • The younger you are when first diagnosed
  • When surgery is an option
  • Your cancer is caught early and is confined to a small area

That’s why it’s vital you seek medical attention as soon as possible, especially if you have been exposed to asbestos, the main cause of mesothelioma.

Mesothelioma staging - TNM

Originally developed by the International Mesothelioma Interest Group, the TNM staging system is the most accurate and the most commonly-used system in mesothelioma diagnosis. It describes tumors (T), lymph nodes (N) and how your cancer has spread, or metasis (M).

The TNM staging system is very detailed, involving notations like:  T1N0MX or T3N1M0 where the tumors, the lymph nodes and the metastasis is further quantified.

The importance of staging

By conducting the appropriate tests, your physician can determine the staging of your mesothelioma. This is important because it:

  • Qualifies how serious your cancer is and your chances of survival
  • Helps your physician plan the best treatment for you
  • Enables your physician to identify clinical trials that may be treatment options for you

Recent statistics on mesothelioma prognosis

As malignant mesothelioma is relatively rare when compared to other cancers, there are not many recent statistics regarding mesothelioma prognosis. On its website, the American Cancer Society cites a large international study of pleural mesothelioma completed in 2009 that revealed the median survival rate using a stage system that was more generally described. These survival rates for malignant mesothelioma ranged from 21 months for Stage I to only 12 months for Stage IV.


Symptoms and Risk Factors With Mesothelioma Doctor Nicholas Vogelzang