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A
Guide to Mesothelioma
What is mesothelioma or Asbestos Cancer?
Mesothelioma is a rare cancer tissue, associated especially
with exposure to asbestos or a cancer that is directly traceable to repeated or long-term
exposure to asbestos. Usually, though, a longer explanation is needed.
The disease gets its name because cancerous tumors
form in the mesothelium – the lining – of an internal organ. In the case of
mesothelioma, the tumors form in the lining of the lung, the lining of the
abdomen, the lining of heart or the lining of a testicle.
Although the average patient
survives only 4 to 18 months after diagnosis, and only 10 percent of patients
survive at least five years, there have been reports of long-term survivors who
have no evidence of disease many years following treatment.
Causes, Risk Factors and Symptoms
Asbestos exposure is
the most common cause of mesothelioma. The term “asbestos”
actually refers to
several minerals with shared properties: long, flexible fibers that are
resistant to stretching, heat, and corrosion. It’s because of these properties
that asbestos was used in products such as insulation, roofing, floor and
ceiling tiles, and brake pads.
Most people present with complaints of shortness of
breath. They also can have complaints of chest pain. Surprisingly, this pain is often
not pleuritic; that is, it does not get worse with deep breathing. This is
surprising in that the pleura (outer surface of the lung) is often involved in
this disease, and most other diseases involving the pleura are often associated
with pleuritic pain (pain that worsens with deep breathing). Patients may also
be asymptomatic, with the disease discovered by physical exam or an abnormal chest X-ray.
As the disease progresses, shortness of breath increases,
and weight
loss, decreased appetite, and night sweats can develop. Local invasion by the
tumor can result in changing of voice, loss of function of the diaphragm, and
symptoms specific to the area and involvement of adjacent structures.
What causes mesothelioma?
Most people with malignant mesothelioma have worked on
jobs where they breathedasbestos. Usually, this involves men over 40
years of age. Others have been exposed to asbestos in a household environment,
often without knowing it. Interestingly, the number of new cases of
mesothelioma has been relatively stable since 1983, the same time that the
restrictions on asbestos were instituted by the U.S. Occupational Safety and
Health Administration (OSHA). In Europe, the number of new cases of
mesothelioma continues to rise.
How much asbestos exposure does it take to get
mesothelioma?
An exposure of as little as one or two months can result
in mesothelioma 30 or 40 years later and in some cases, as much as 70 years
later.
How long does it take after asbestos exposure
for mesothelioma to show up?
People exposed in the 1940s, '50s, '60s, and '70s are now
being diagnosed with mesothelioma because of the long latency period of
asbestos disease.
Mesothelioma is diagnosed by pathological examination from a
biopsy. Tissue is removed, placed under the microscope, and a pathologist makes
a definitive diagnosis and issues a pathology report. This is the end of a
process that usually begins with symptoms that send most people to the doctor:
a fluid buildup around the lungs (pleural effusions), shortness of breath, pain in the chest, or
pain or swelling in the abdomen. The doctor may order an X-ray or CT scan of the chest or abdomen. If further examination
is warranted, the following tests may be done:
Thoracoscopy For pleural
mesothelioma
Peritoneoscopy For peritoneal
mesothelioma
Biopsy If abnormal tissue
is found
While asbestos is the
most common cause of mesothelioma, certain characteristics of the disease
suggest that asbestos exposure is not the only cause. Other suspects include:
Simian Virus 40
Is an abbreviation for Simian vacuolating virus 40 or Simian
virus 40, a polyoma virus that is found in both monkeys and humans. Like other polyoma viruses, SV40 is a DNA virus that has the potential
to cause tumors, but most often persists as a latent
infection.
SV40 became a highly
controversial subject after it was revealed that millions were exposed to the
virus after receiving a contaminated polio vaccine produced between 1955 and
1961.
Causes & Risk Factors of Simian 40
BACKGROUND:
The presence of SV40 in monkey cell cultures used in the
preparation of the polio vaccine from 1955 through 1961 is well documented.
Investigations have consistently demonstrated the oncogenic behavior of SV40 in
animal models. Early epidemiologic studies were inadequate in demonstrating an
increase in cancer incidence associated with contaminated vaccine. Recently,
investigators have provided persuasive evidence that SV40 is present in human ependymomas,
choroid plexus tumors, bone tumors, and mesotheliomas, however, the etiologic
role of the virus in tumorigenesis has not been established.
MATERIALS AND
METHODS:
Using data from SEER, we analyzed the incidence of brain
tumors, bone tumors, and mesotheliomas from 1973-1993 and the possible
relationship of these tumors with the administration of the SV40 contaminated
vaccine.
RESULTS:
Our analysis indicates increased rates of ependymomas (37%),
osteogenic sarcomas (26%), other bone tumors (34%) and mesothelioma (90%) among
those in the exposed as compared to the unexposed birth cohort.
CONCLUSIONS:
These data suggest that there may be an increased incidence
of certain cancers among the 98 million persons exposed to contaminated polio
vaccine in the U.S.; further investigations are clearly justified.
Erionite
Erionite is a naturally
occurring fibrous mineral that belongs to a group of minerals called zeolites. It usually is found
in volcanic ash that has been altered
by weathering and ground water. Erionite forms
brittle, wool-like fibrous masses in the hollows of rock formations and has an
internal molecular structure similar to chabazite. Some properties of
erionite are similar to the properties ofasbestos; however, erionite is
not currently regulated by the U.S. Environmental Protection Agency as one of the six
asbestos fibers. Erionite was
first described by A.S. Eakle in 1898, as white woolly fibrous masses in
cavities in rhyolite lava near Durkee,
Oregon. It was originally
thought to be another relatively rare zeolite named offretite, which is very
similar to erionite in appearance and chemical composition.
Genetic Predisposition
Three villages in Turkey provide an example of how genetic
factors can play a role in mesothelioma development. In the villages of Tuzkoy,
Karain and "Old" Sarihidir, where approximately 50 percent of all
deaths are caused by malignant mesothelioma, some families have higher
incidences of the cancer even though erionite
exposure levels are the same for other
families in the area. The initial suggestion from researchers studying this
occurrence was that a unique and more carcinogenic form of erionite was present
in certain houses, but an evaluation of the structure of erionite from
different homes proved the fiber structures were similar.
Additional studies showed that when high-risk mesothelioma
family members married into families with no history of the disease, their
children developed mesothelioma. This finding – along with subsequent studies
that found more evidence of genetic predisposition – has convinced many
researchers that inherited genetics is responsible for higher susceptibility to
erionite exposure and mesothelioma in the region. Although the relationship
between genetic susceptibility and mesothelioma is still being investigated,
the situation in Turkey presents a strong argument for genetics playing a role
in mesothelioma cancer.
Some of these
occupations are:
Insulation work in
buildings and other structures
Demolition work,
especially in older buildings
Drywall workers
Firefighters
Automobile mechanics
Steel mill workers
Shipyard and
shipbuilding trades
Navy veterans Family
members of asbestos-exposed workers may develop mesothelioma because of regular
exposure to the fibers brought home on clothing, shoes, and hair. Regular
exposure may have occurred from tasks such as washing work clothes that
contained asbestos fibers.
BAP1 Gene
Recent research unveiled a new gene that may play a
significant role in a person's likelihood of developing mesothelioma. In a 2011
study conducted by researchers at the University of Hawaii Cancer Center and Fox Chase Cancer Center in Philadelphia, Pennsylvania, it was discovered that
people who carry a mutation in a gene called BAP1 are susceptible to developing
mesothelioma. When these individuals are exposed to asbestos or a similar
mineral like erionite, their risk for contracting mesothelioma is significantly
increased.
The study evaluated two U.S. families with high incidences
of mesothelioma and the mutation of the BAP1 gene. Researchers noticed that
every family member who had contracted mesothelioma also carried the BAP1
mutation.
Further investigation into 26 individuals with
mesothelioma without a family history of the cancer confirmed these results,
with 25 percent of those studied carrying mutations of the BAP1 gene.
Genes Associated with Mesothelioma
Researchers were first inclined to study genetics as a risk factor for mesothelioma after the cancer was found more commonly among family
members in the 1960s. Since, many studies have been conducted and researchers
have identified some genes that may play a role in the development of
mesothelioma.
For example, in one study of a family of three sisters
with malignant mesothelioma and one brother with pleural plaques — who were all
exposed to asbestos by living near an asbestos cement factory — the
siblings lacked a chromosomal structure at the 9p gene region. This gene –
known as a tumor suppressor – is related to cancer suppression when
present, and the lack of it enables tumor growth and the development of mesothelioma.
Other tumor suppressor genes that may affect the development of mesothelioma
include P16INK4a, P16/CDKN2A, P14ARF and neurofibromatosis 2 (NF2).
Two other genes that have been commonly associated with
mesothelioma include glutathione-S-transferase M1 (GSTM1) and
N-acetyltransferase (NAT2). GSTM1 is important in the detoxification of
carcinogens such as asbestos, and NAT2 is involved in the biotransformation of
carcinogens. The absence of GSTM1 can result in a twofold increase in risk for
mesothelioma and the absence of both GSTM1 and NAT2 increases a person's risk
sevenfold.
Diagnosis & Staging
Doctors utilize staging systems to better diagnose
potential treatment options for patients. Depending on the stage and nature of
the cancer, different surgery and chemotherapy options may be available. An internationally accepted
staging system allows doctors to standardize the diagnosis of mesothelioma,
which helps medical professionals research treatment procedures that are more
beneficial to the patient.
Because of the need for an internationally accepted
staging system, three systems of diagnosing malignant mesothelioma were
developed. The majority of doctors tend to use the TNM system. While these
staging systems are used primarily for pleural
mesothelioma, there is currently no formal
staging system for the other types of this cancer.
Because mesothelioma is
a rare cancer, it requires disease-specific expertise. Here are some places
you can go to find a mesothelioma specialist:
Local hospital referral
services
Your nearest National
Cancer Institute designated cancer center (search by state at
American Board of
Medical Specialties at www.abms.org
American Medical
Association’s DoctorFinder at webapps.ama-assn.org/
doctorfinder/home.jsp
American Society of
Clinical Oncology’s patient site at www.cancer.net
American College of
Surgeons at www.facs.org/patienteducation
Finding a Specialist
Prepare for your
appointment by formulating a list of questions to ask. You may want to divide
questions into two categories:
What to Ask the Doctor
Do you have experience
treating mesothelioma?
How long have you been
in practice, and how many cases of mesothelioma have you treated?
What types of treatment
do you usually recommend?
How many times have you
performed the surgery I need, and what’s your success rate?
Are you involved in
clinical trials and research?
Who else will be on my
health care team, and what are their qualifications?
What to Ask Yourself
Did the doctor give you
a chance to ask questions?
Did you feel like the
doctor was listening to you?
Did the doctor seem
comfortable answering your questions?
Did the doctor talk to
you in a way you could understand?
Do you feel the doctor
respected you?
Did the doctor ask your
preferences for treatment?
Questions to Ask
Improvements in
treatment have been made in recent years, giving mesothelioma patients hope. An
overview of current and investigational therapies are detailed here.
Surgery
Cutting out tumors, along with chemotherapy, is considered
the most effective way to eliminate cancer from your body. This is particularly
true with pleural mesothelioma and peritoneal mesothelioma. An increasing
number of surgeons are performing surgery to remove cancer from the lining of
the lungs or from the lining of the abdominal cavity.
Surgeries can have different objectives, depending upon
the stage of the cancer and your overall health. Some can be highly invasive
and lengthy, but potentially curative and life changing. Others can be less
traumatic and more palliative in nature. Surgery often is accompanied by some type
of chemotherapy and radiation therapy, with the goal of killing any cancer
cells that might have been left behind.
When an individual is diagnosed with cancer, treatment options
will be one of the first topics discussed with the oncologist. The available
treatment options will vary from patient to patient depending on the type of
mesothelioma that has been diagnosed and the stage that the disease has
progressed to. Mesothelioma, in particular, is a very serious and aggressive
form of cancer for which treatment options have traditionally been quite
limited.
When a recently-diagnosed mesothelioma patient is presented with options for
treatment, surgery may be recommended. There are different forms of
mesothelioma surgery and some are proposed as a curative measure, while others are
designed to relieve symptoms of the disease that can be quite debilitating and
interfere with the patient's quality of life.
Curative Surgeries
Due to the nature of mesothelioma and the fact that by the time a diagnosis is made the disease has usually
reached an advanced stage, a surgical cure is not a likely option. However, in
some cases, surgery is suggested in order to remove as much of the cancer as
possible and is often followed by other mesothelioma treatments such as chemotherapy and/or radiation.
Ideally, candidates for these surgeries will be in the early
stages of the disease where the tumor is still fairly localized and metastasis
has not occurred. In addition, the patient will need to be in overall good
health and free of other medical issues that can complicate these procedures
and lengthen the recovery period. Extant medical issues - like heart disease,
hypertension, and diabetes - are often an issue with older mesothelioma
patients, further limiting treatment options and making aggressive surgical
procedures a less favorable choice.
Treatment Options
Advances
in the treatment of mesothelioma offer hope for anyone affected by this rare
cancer.
Today, some survivors live five to 10 years or more after
receiving a mesothelioma diagnosis -- a life expectancy that once was measured
by months. Learn about your body and this disease, and take control of your
cancer treatment.
While there is no absolute cure for this asbestos-related
cancer, potentially curative treatment plans exist so long as the disease is
diagnosed early enough and medical care is specialized. Because mesothelioma is
such a complicated disease, treatment should be tailored to exactly what your
body needs. That means factoring in the stage and type of your cancer as well
as your age, general health and how aggressive you want your treatment to be.
Types of Mesothelioma Treatment
The types of treatment you can receive depend on your
diagnosis. Treatment is typically broken down into two categories: curative and
palliative. Curative treatments like surgery aim to get cancer out of your
body. If your tumors do not metastasize, you should have surgical options. In
addition, different chemotherapy regimens and diverse radiation treatments can
help. Palliative treatments aim to make you more comfortable as you live with
cancer. Doctors will address your symptoms with the goal of improving the
quality of your life.
In both cases, mesothelioma patients should ask about
options other than the standards of surgery, chemotherapy and radiation
therapy. Experimental treatments do exist, mostly through clinical trials. They
include rapidly developing gene therapy and immunotherapy, along with
photodynamic therapies, all of which can be discussed with an experienced
specialist.
Emerging and Experimental Treatments
Because of the low rate of successful
treatments today, the future of mesothelioma treatment almost certainly
involves therapies still in development. Do not be afraid to bring this up for
discussion with your specialists. The potential of immunotherapy, gene therapy
and photodynamic therapy is enormous, and research on these new treatments is
considered cutting edge.
Immunotherapy
This involves manipulating your own
immune system to help fight off the cancer growth. This is a specific treatment
that involves your own cells being altered in the laboratory and returned to
your body to fight the cancer without the use of toxic drugs.
Gene Therapy
This involves repairing the defective
genes by injecting the patient with a modified virus to do the work. Regardless
of the location of the cancer, gene therapy can introduce genetic material that
targets the cancer cells.
Photodynamic Therapy
This uses light energy to kill the
mesothelioma cells. A drug makes the cancer cells sensitive to a particular
light wavelength, and then the tumor cells are exposed to the light through a
laser. It is an outpatient procedure with few harmful side effects.
Legal Issues
Statutes of limitations
vary from state to state and may limit the time in which you can file a lawsuit
after diagnosis. The limitation period can be as short as one year or as long
as three years or more, but the sooner you start the legal process, the sooner
you will receive compensation to help with medical bills and other expenses.
Your case may be expedited depending on your symptoms and the stage of the
disease.
The most common types
of lawsuits include:
Personal injury: These lawsuits are
filed for personal suffering and pain associated with the illness, which can
occur from direct or secondhand exposure to asbestos. Compensation is based on
the degree of incapacity, loss of income, and quality of life related to
mesothelioma symptoms and treatment. Damages are awarded for medical expenses,
lost wages, lost prospects, travel expenses for treatment, pain, and suffering.
Wrongful death: This type of lawsuit is
filed on behalf of the family after the patient’s death. The damages awarded
are for payment of mesothelioma-related medical bills, lost income, counseling
related to the patient’s death, legal advice, personal loss, and other
illness-related services. Choosing an attorney is a personal decision, just as
when choosing a medical team. You should feel comfortable with the answers you
receive to your questions, the way you are treated by the attorney and staff,
the level of experience and positive outcomes the law firm has had with other mesothelioma
and asbestos-related cases, and the firm’s reputation.
An attorney should
never promise you a specific amount of compensation. There are no guarantees;
each case is unique. Legal advice and the process of filing a lawsuit is an
expensive undertaking. Most attorneys accept a case based on a contingency fee,
meaning you will have no out-of-pocket expenses and the attorney will receive a
percentage of your settlement money as payment. The contingency fee pays for
legal work done on your behalf, court filing fees, expert witness fees, and
legal representation in court. If an award is not granted, you may be
responsible for some expenses depending on the contingency fee agreement. Once
you decide to hire an attorney, you will meet with the legal staff to provide information
about your work history and personal history to determine the type and degree
of asbestos exposure you sustained. Authorizations will be obtained to get
copies of your medical records, and you should not have to undergo any further
medical testing for the purposes of the lawsuit. Next, you will be asked to
give a deposition (getting your testimony on tape) in case you are not able to
appear in court. Some attorneys also have a videographer follow you for a day
to document what a typical day with mesothelioma is like for you. This can be shown
in court to support your case. Many patients worry that with a lengthy litigation
process, they may die before the case is settled. If you pass away, your loved ones
can continue to litigate in your place if you have designated an executor of
your estate and beneficiaries in your will.
End of life care focuses
on patients’ comfort and symptom relief, while incorporating spiritual and
psychological counseling to help prepare for a good death.
Preparing for death
also involves drawing up legal documents, such as a health care power of
attorney and living will. With a health care power of attorney, you will want
to choose a person to make medical decisions and initiate your care plan when
you are no longer able to speak for yourself. Sometimes close family members
are not able to separate emotions from logic and may have difficulty carrying out
your wishes, so discuss your wishes with your appointee to verify his/her cooperation
and comfort with serving as your health care power of attorney. A living will,
sometimes referred to as an advance directive, defines what, if any, extraordinary
measures a person would want to be taken to prolong his or her life. Many of
the documents you’ll need can be found at www.caringinfo.org, a website run by
the National Hospice and Palliative Care Organization. Hospice services are
designed to provide symptom relief, pain management, and support to patients
and their families. A person is referred to hospice services when life
expectancy is less than six months. Most hospice services take place in
patients’ homes, assisted living facilities, or nursing homes. Depending on
where you live, you may have multiple options when choosing a hospice provider.
Just as carefully as you chose the health care team to treat your mesothelioma,
you will want to do the same in selecting a hospice provider.
Set up an
interview and start by asking the following questions:
Does the provider serve
the area in which you live?
How long has the
provider been in operation?
Does Medicare certify
the provider?
Does the provider
accept Medicaid?
Does your insurance
cover the provider?
Is the provider
licensed by the state?
What additional
services does the provider cover, such as follow-up phone calls, mailings,
support groups, and retreats or activities for families?
What services, if any,
are not provided?
Is participation in
care by a family member required for enrollment in services?
What is expected from
the family caregiver?
What, if any,
out-of-pocket charges can be expected?
How flexible is the
schedule of visits?
Who provides the
on-call coverage?
Does the provider have
a residential facility?
What types of services
are provided for funeral arrangement support?
How are bereavement
services managed, and how long do they last?
After answering your
questions, if you are still uncertain whether the provider is right for you,
you can request an evaluation visit. This will give you the chance to interact
with the staff in a comfortable environment and get a better idea of how the
services will work. You may also want to research the reputation of the agency
by asking your doctor, a chaplain, other health care professionals, and families
of previous patients for advice and information.
Information
presented is not intended as a substitute for the personalized professional advice
given by your health care provider. Although great care has been taken to
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