a patient’s Guide
What Is Mesothelioma? n Causes and Risk Factors n Finding a
Specialist n Treatment Options n Legal Decisions n Resources
C a n C e r U p D at e S , r e S e a r C h & e D U C at I o n
Based on science, but filled with humanity,
CURE makes cancer understandable.
a patient’s Guide
MeSotheLIoMa IS a rare CanCer, with 2,000 to 3,000 new cases
diagnosed each year, and understanding the disease is impor tant in
making decisions about care and treatment.
MeSotheLIoMa ForMS in the mesothelium, worked in shipyards or as miners and are
a protective membrane covering internal men age 60 and older, but family members
organs, such as the lungs and heart. The of these workers have also developed the
mesothelium is made up of two layers—one disease—many with relatively little direct
surrounding the organ itself and the other exposure. Also, because mesothelioma can
forming an outer sac. Between the layers is a take decades to develop, most patients are
lubricating fluid that lessens friction between older at diagnosis.
the membranes and permits the organs to The geographic distribution of this disease
glide smoothly against each other. This is is not uniform across the United States,
particularly important to the heart and lungs,
with the highest death rates appearing in
because they produce significant movement
the Northeastern states, along the Pacific
as they function.
Coast, and in Illinois, Florida, Wyoming, and
Mesothelioma is defined by the site of
Colorado. As with the demographic imbalance,
the primary tumor. Pleural mesothelioma, in
the geographic distribution can also be
which cancer cells surround the lungs and/or
explained by asbestos exposure—the regions
the chest wall, accounts for about 85 percent
with the highest death rates are (or were)
of malignant mesotheliomas. Mesothelioma
home to either factories that manufactured
can also grow across the thin mesothelial
tissue that lines the abdomen (peritoneum) asbestos-containing products or shipbuilding
or, rarely, the heart or testicles. facilities (asbestos materials were commonly
Mesothelioma is different from other used as insulation in ships).
cancers because it’s tightly linked with an Although the average patient survives only
environmental cause—asbestos exposure, four to 18 months after diagnosis, and only
which accounts for the majority of cases. 10 percent of patients survive at least five
Most mesothelioma patients worked or years, there have been reports of long-term
lived in a place where they were exposed survivors who have no evidence of disease
to asbestos. Typical mesothelioma patients many years following treatment.
Causes & risk Factors
aSBeStoS expoSUre IS the MoSt CoMMon method by which asbestos fibers arrive at the
cause of mesothelioma. the term “asbestos” mesothelium is not certain, it is likely that this
actually refers to several minerals with shared journey takes years, a belief that fits with the
properties: long, flexible fibers that are extended period of time necessary to develop
resistant to stretching, heat, and corrosion. It’s
It has never been established if there
because of these properties that asbestos was
are any “safe” levels of asbestos exposure.
used in products such as insulation, roofing,
However, there is at least circumstantial
floor and ceiling tiles, and brake pads.
evidence that even low environmental levels
Though the asbestos industry launched in of asbestos exposure can increase the risk
the late 1800s, scientists didn’t link the of mesothelioma.
mineral to mesothelioma until the 1950s. In Interestingly, smoking is not a risk factor
the United States, asbestos continued to be for mesothelioma. However, smoking does
mined and used—particularly for insulation— increase the risk of lung cancer, which is
until the 1980s. frequent in people with asbestos exposure.
Of the various types of asbestos, the fibers Thus, people with both a history of smoking
most commonly used in the United States are and asbestos exposure have an elevated risk
chrysotile (white asbestos), crocidolite (blue of developing lung cancer—approximately
asbestos), and amosite (brown asbestos). 60-fold higher than nonsmokers with no
Chrysotile is most widely used because its asbestos exposure.
fibers are curled, a property that makes it While asbestos is the most common
less likely to fragment than the other types of cause of mesothelioma, certain
asbestos. Crocidolite and amosite are typically characteristics of the disease suggest that
used in limited, specialized applications asbestos exposure is not the only cause.
because their needle-like structure makes Other suspects include:
them highly susceptible to fragmenting. This
is important because if asbestos is easily SIMIan VIrUS 40
crushed, it remains airborne longer, which Due to conflicting research, perhaps the
increases the likelihood of inhaling the fibers most controversial link to mesothelioma is
that cause mesothelioma. exposure to simian virus 40 (SV40), a virus
Generally, it takes 20 to 40 years or that contaminated polio vaccines during the
more for mesothelioma to develop following 1950s and 1960s. In the ’50s, monkeys were
asbestos exposure. The exact means by which used to develop a vaccine against polio, but in
asbestos causes cancer has not yet been fully 1961, it was discovered that these monkeys
determined, but it is known when asbestos were infected with SV40, which meant the polio
is inhaled, about two-thirds of the fibers are vaccines were likely contaminated. Shortly
eliminated from the body by coughing or thereafter, animal studies showed that SV40
swallowing. The remaining fibers travel through was capable of causing cancer, producing
the airways, and some may reach the alveoli, significant alarm regarding the cancer-causing
the tiny air sacs in the lungs. Although the potential of the contaminated polio vaccines.
Who’s at Risk?
CanCer patients often wonder why they got
the disease. Most cases of mesothelioma
involve asbestos exposure, but you may be
unsure when, or even how, your exposure
Some research supports the possibility occurred. Although minute levels of asbestos
that SV40 and asbestos may work together exist in the air, water, and soil, most people
to cause mesothelioma. For instance, cells
do not develop mesothelioma from this type
with chromosomal abnormalities (such as
of exposure. People who develop the disease
those caused by asbestos exposure) can
generally have regular asbestos exposure,
gain cancer-like characteristics more easily
when infected with SV40 compared to cells such as from a job.
with normal chromosomes. In the 1970s, legislation was enacted to
protect workers from asbestos exposure, but
erIonIte prior to that, workers in certain industries may
Erionite is a non-asbestos mineral fiber have had regular exposure to asbestos. Some
that is similar in physical characteristics to
of these occupations are:
crocidolite. Erionite has been identified as
a cause of mesothelioma based on studies n Insulation work in buildings and other
of mesothelioma rates among residents of
Cappadocia, Turkey, where erionite has been
n Demolition work, especially in older
used in building materials. Of 141 deaths
in four villages during a four-year period, 24 buildings
percent were due to mesothelioma. Erionite n Drywall workers
deposits can be found throughout the western n Firefighters
United States, primarily in areas with a low n Automobile mechanics
population density. To date, however, there have n Steel mill workers
been no cases of mesothelioma attributed to
n Shipyard and shipbuilding trades
erionite exposure in the United States.
n Navy veterans
GenetIC preDISpoSItIon Family members of asbestos-exposed
Further examination of the link between
workers may develop mesothelioma because
erionite exposure in Turkey and the high
of regular exposure to the fibers brought home
mesothelioma death rate produced evidence
that genetic predisposition might also be on clothing, shoes, and hair. Regular exposure
a factor in development of the disease. may have occurred from tasks such as washing
Although all homes in that region contained work clothes that contained asbestos fibers.
similar amounts of erionite, mesothelioma
occurred in some homes and not in others.
In addition, mesothelioma development of the parents causing the disease. While
followed a dominant genetic inheritance these observations cannot be considered
pattern, in which half of the children of conclusive evidence for a genetic
parents with mesothelioma also developed predisposition to mesothelioma, they do
mesothelioma. This pattern is consistent provide reason to further study the role of
with a single genetic mutation from one genetics as a risk factor.
Diagnosis & Staging
DIaGnoSInG MeSotheLIoMa can be a Radiological analysis using contrast-
complicated process. Symptoms do not enhanced computed tomography (CT)
typically arise until the disease has reached scan provides a three-dimensional view
the late stages, and when they do arise, most of the entire pleural surface. It can help
determine the extent of disease and can aid
are nonspecific, including weight loss, fatigue,
the physician in finding the desired tissue
and night sweats.
for biopsy. Magnetic resonance imaging
Depending on the type of mesothelioma, (MRI) and positron emission tomography
organ-specific symptoms may be present. (PET) scans may also be used to diagnose
For instance, most patients with pleural mesothelioma.
mesothelioma experience lung-related Once the diagnosis is made, the disease
symptoms, such as consistent chest pain stage is evaluated. As with any type of
and progressively worsening shortness cancer, determining the extent of the disease
of breath. The pain is typically caused by is used to predict a patient’s outcome and to
invasion of cancer into the chest wall, and determine the best course of treatment.
shortness of breath is often caused by In stage 1, the disease is confined to
pleural effusion (excess fluid in the pleural the mesothelium and has not spread to the
linings of the lungs). The presence of cough, lymph nodes or any other organs. Stage 1A
or coughing up blood, can occasionally occur refers to a tumor in the outer layer of the
with pleural mesothelioma. mesothelium, whereas stage 1B refers to a
Proper diagnosis requires a combination tumor that has reached both the outer and
of physical, radiological, and pathological inner layer. Stage 1 is the most treatable,
examinations, as well as an accurate and patients with this stage disease have the
patient history, including potential asbestos best prognosis (expected outcome).
exposure. Stage 2 disease is characterized by the
For patients with pleural effusions, a spread of cancer beyond the mesothelium
sample of the fluid is examined for the and into the lung tissue and/or diaphragm,
presence of malignant cells. In most cases, but not yet into the lymph nodes. Patients
even when a pleural fluid sample is available, with stage 2 mesothelioma may be
a biopsy of the pleural tissue is required to candidates for surgical removal of the tumor.
make a diagnosis. Examination of the biopsy For stage 3 mesothelioma, the cancer has
samples includes immunohistochemistry (IHC) spread into the fatty part of the mediastinum
tests, in which the samples are processed (the space behind the breastbone and
in a way that allows the pathologist to between the lungs) and/or to the lymph
look for specific signs, called markers, of nodes within the thorax.
mesothelioma. There are no known markers Stage 4 is metastatic disease, where the
that correspond to mesothelioma alone, cancer has spread to other organs, such as
so in order to diagnose mesothelioma, the the heart or opposite lung. The cancer may
pathologist generally evaluates the presence also have spread into the peritoneum, to the
or absence of several markers. lymph nodes, or into the spine.
Questions to Ask
Finding a prepare for your appointment by formulating
a list of questions to ask. You may want to
Specialist divide questions into two categories:
What to aSk the DoCtor
BeCaUSe MeSotheLIoMa IS a rare CanCer, n Do you have experience treating
it requires disease-specific expertise. mesothelioma?
n How long have you been in practice, and
Here are some places you can go to find a how many cases of mesothelioma have you
mesothelioma specialist: treated?
n What types of treatment do you usually
n Local hospital referral services
n Your nearest National Cancer Institute-
n How many times have you performed the
designated cancer center (search by state at
surgery I need, and what’s your success
n Are you involved in clinical trials and
n American Board of Medical Specialties at
www.abms.org n Who else will be on my health care team,
n American Medical Association’s and what are their qualifications?
DoctorFinder at webapps.ama-assn.org/
doctorfinder/home.jsp What to aSk YoUrSeLF
n American Society of Clinical Oncology’s n Did the doctor give you a chance to ask
patient site at www.cancer.net questions?
n American College of Surgeons at n Did you feel like the doctor was listening to
n Mesothelioma Applied Research n Did the doctor seem comfortable
Foundation at www.marf.org answering your questions?
n Did the doctor talk to you in a way you
Once you have found several doctors could understand?
who seem like a good fit, call and ask a n Do you feel the doctor respected you?
few questions—does the doctor take your n Did the doctor ask your preferences for
insurance, is he or she board certified— treatment?
before scheduling an appointment to meet
and interview the doctor/surgeon. oncologists and specialists. Keep in mind
You may also want to get a second that these consultations can be expensive
opinion. Most oncologists expect their and may not be covered by insurance.
patients to seek second opinions, and many For some insurance plans, second
suggest it themselves. If your doctor doesn’t opinions are covered or even required before
recommend a specialist, you can use the the plan approves payment for treatment, but
resources listed to find a doctor who can you may be limited to certain physicians or
offer a second opinion. hospitals.
Nurse coordinators at large medical After you weigh your options, you must
centers can be particularly helpful in finding choose your medical team. The comfort level,
physicians who give second opinions. treatment options proposed, and medical
Support groups and other cancer survivors expertise should all be evaluated when
may also be good sources for recommending making a decision.
IMproVeMentS In treatMent have been This procedure, which can be performed by
made in recent years, giving mesothelioma VATS or through a larger incision on the side
patients hope. an overview of current and of the chest, is associated with a number
of postoperative complications, including air
investigational therapies are detailed here.
leak, bleeding, and pus formation. Conflicting
evidence exists regarding the impact of P/D,
with some studies suggesting it prolongs life
Although complete surgical removal of the
and others indicating it does not.
tumor is desired, this is rarely achievable for
most patients because microscopic tumor n extrapleural pneumonectomy (epp),
tissue is generally left behind. However, the most aggressive surgical treatment
surgery is still used to treat patients with the for malignant mesothelioma, involves the
goal of removing the vast bulk of the tumor removal of both layers of the pleura along
and thus alleviating symptoms. The surgical with the involved lung, some of the lymph
procedures include: nodes, the diaphragm, and the pericardium
(the latter two are reconstructed with
n Video-assisted thoracoscopic (VatS) talc
synthetic materials). Although EPP has
pleurodesis is primarily used to treat pleural
inherent dangers, it permits the use of
effusions, but a biopsy can be obtained at the
radiation therapy following surgery since the
same time for diagnostic purposes. With VATS
involved lung has been removed. Evidence
talc pleurodesis, a camera is inserted into the
suggests radiation after surgery may reduce
pleura through a small incision in the chest,
recurrence at the original tumor site and
and surgical instruments are inserted through
prolong survival in patients with early-
two other incisions. The camera allows the
stage disease. Chemotherapy is now often
surgeon to maneuver instruments into the
incorporated into the treatment plan as
pleural space, where the surgeon first drains
well, either before or after surgery. However,
the excess fluid and then blows talc (similar
because of the morbidity and mortality
to common talcum powder, but sterilized),
associated with this procedure, EPP is only
causing an inflammatory reaction that literally
performed on patients with early-stage
glues the lung to the inner surface of the rib
disease, good cardiac health, no history
cage. Eventually, scar tissue forms, creating
of certain cardiac or lung surgeries, no
permanent bonds that eliminate the space
significant kidney or liver disease, and little,
in which fluid can develop. This procedure
if any, chest pain.
should relieve the shortness of breath that
accompanies pleural effusions.
n pleurectomy/decortication (p/D) is Radiation therapy is generally used to treat
performed with the goal of removing as much localized patches of tumor recurrence
of the tumor as possible by removing part of or small areas of tumor left behind after
the pleura as well as the fibrous covering of surgery. There is no evidence to suggest
the lungs, which may restrict lung expansion. radiation alone improves survival, but it does
alleviate pain for approximately half of the experimental drug Onconase (ranpirnase),
patients receiving it. showed the average survival of both groups
was nearly identical: 10.7 months for the
CheMotherapY Onconase group versus 11.1 months for
The goals of current chemotherapy regimens patients receiving Adriamycin alone. However,
are to prolong the survival of mesothelioma when investigators looked only at patients
patients and improve their quality of life by who had already received one course of
relieving disease-related symptoms. chemotherapy, they found that those patients
The approval of Alimta (pemetrexed) in survived longer, on average, when Onconase
2004 has led to the largest improvement in was added to treatment: 10.5 months for
patient outcomes. When added to cisplatin, the Onconase group versus nine months for
Alimta, which works by inhibiting cellular patients receiving Adriamycin alone.
proteins that stimulate cancerous cells to Also presented at ASCO’s annual meeting
grow, was shown in a clinical study to prolong were results from studies with the targeted
average survival by almost three months. agents cediranib and NGR-hTNF, both of
Alimta also relieved pain and shortness which target the tumor’s blood supply.
of breath, and improved lung function in Cediranib and NGR-hTNF were examined
some patients. Alimta plus cisplatin given as single agents, and both demonstrated
intravenously once every three weeks has the ability to slow disease progression in
become standard therapy for newly diagnosed patients with relapsed mesothelioma. As a
mesothelioma. (Clinical studies of Tomudex result of these findings, new clinical trials are
[raltitrexed], a drug in the same class as being planned.
Alimta, have also shown improved response An ongoing phase III trial is investigating
rates and survival times when given with Zolinza (vorinostat), which is already
cisplatin compared with cisplatin alone.) approved for a rare type of lymphoma, to
Premedication with folic acid and vitamin see if it improves survival of patients with
B12 is necessary when taking Alimta in mesothelioma. In this trial, patients whose
order to reduce the frequency of side effects, disease progressed after treatment with
which can include fatigue, nausea, vomiting, Alimta will be given either Zolinza or placebo.
anorexia (loss of appetite), constipation, New treatments in phase II development
mouth sores, and low blood cell counts. More include drugs approved by the Food and Drug
severe side effects, including renal failure and Administration for other cancers—Sprycel
hearing loss, occur in rare instances. (dasatinib), Velcade (bortezomib), Sutent
(sunitinib), Afinitor (everolimus), and Eloxatin
InVeStIGatIonaL therapIeS (oxaliplatin)—as well as investigational
A number of important mesothelioma drugs that are not approved (pazopanib,
studies were presented at the 2009 annual vandetanib, and vatalanib).
meeting of the American Society of Clinical
Oncology. A phase III trial comparing Find ongoing mesothelioma clinical trials at
Adriamycin (doxorubicin) with or without the www.curetoday.com/trialcheck.
CoMpanIeS InVoLVeD In the ManUFaCtUre outcomes the law firm has had with other
and distribution of products containing asbestos mesothelioma and asbestos-related cases,
and the firm’s reputation. A word of caution:
can be held liable for the development of
An attorney should never promise you a
mesothelioma in workers exposed to asbestos.
specific amount of compensation. There are
Statutes of limitations vary from state no guarantees; each case is unique.
to state and may limit the time in which Legal advice and the process of filing
you can file a lawsuit after diagnosis. The a lawsuit is an expensive undertaking.
limitation period can be as short as one Most attorneys accept a case based on a
year or as long as three years or more, but contingency fee, meaning you will have no
the sooner you start the legal process, the out-of-pocket expenses and the attorney will
sooner you will receive compensation to help receive a percentage of your settlement money
with medical bills and other expenses. Your as payment. The contingency fee pays for legal
case may be expedited depending on your work done on your behalf, court filing fees,
symptoms and the stage of the disease. expert witness fees, and legal representation
The most common types of lawsuits include: in court. If an award is not granted, you may
n personal injury: These lawsuits are filed be responsible for some expenses depending
for personal suffering and pain associated on the contingency fee agreement.
with the illness, which can occur from direct Once you decide to hire an attorney, you
or secondhand exposure to asbestos. will meet with the legal staff to provide
Compensation is based on the degree of information about your work history and
incapacity, loss of income, and quality of personal history to determine the type and
life related to mesothelioma symptoms and degree of asbestos exposure you sustained.
Authorizations will be obtained to get copies
treatment. Damages are awarded for medical
of your medical records, and you should not
expenses, lost wages, lost prospects, travel
have to undergo any further medical testing
expenses for treatment, pain, and suffering.
for the purposes of the lawsuit.
n Wrongful death: This type of lawsuit
Next, you will be asked to give a deposition
is filed on behalf of the family after the
(getting your testimony on tape) in case
patient’s death. The damages awarded are
you are not able to appear in court. Some
for payment of mesothelioma-related medical
attorneys also have a videographer follow
bills, lost income, counseling related to the
you for a day to document what a typical day
patient’s death, legal advice, personal loss,
with mesothelioma is like for you. This can be
and other illness-related services.
shown in court to support your case.
Choosing an attorney is a personal Many patients worry that with a lengthy
decision, just as when choosing a medical litigation process, they may die before the
team. You should feel comfortable with the case is settled. If you pass away, your loved
answers you receive to your questions, the ones can continue to litigate in your place
way you are treated by the attorney and if you have designated an executor of your
staff, the level of experience and positive estate and beneficiaries in your will.
end of Life
enD oF LIFe Care FoCUSeS on patients’ start by asking the following questions:
comfort and symptom relief, while incorporating n Does the provider serve the area in which
spiritual and psychological counseling to help you live?
prepare for a good death. n How long has the provider been in
Preparing for death also involves drawing up
n Does Medicare certify the provider?
legal documents, such as a health care power
n Does the provider accept Medicaid?
of attorney and living will. With a health care
n Does your insurance cover the provider?
power of attorney, you will want to choose a
person to make medical decisions and initiate n Is the provider licensed by the state?
your care plan when you are no longer able n What additional services does the provider
to speak for yourself. Sometimes close family cover, such as follow-up phone calls,
members are not able to separate emotions mailings, support groups, and retreats or
from logic and may have difficulty carrying activities for families?
out your wishes, so discuss your wishes with n What services, if any, are not provided?
your appointee to verify his/her cooperation n Is participation in care by a family member
and comfort with serving as your health care required for enrollment in services?
power of attorney. n What is expected from the family
A living will, sometimes referred to as caregiver?
an advance directive, defines what, if any, n What, if any, out-of-pocket charges can be
extraordinary measures a person would expected?
want to be taken to prolong his or her life. n How flexible is the schedule of visits?
Many of the documents you’ll need can be n Who provides the on-call coverage?
found at www.caringinfo.org, a website run n Does the provider have a residential
by the National Hospice and Palliative Care facility?
Organization. n What types of services are provided for
Hospice services are designed to provide funeral arrangement support?
symptom relief, pain management, and n How are bereavement services managed,
support to patients and their families. A and how long do they last?
person is referred to hospice services when
life expectancy is less than six months. Most After answering your questions, if you are
hospice services take place in patients’ still uncertain whether the provider is right for
homes, assisted living facilities, or nursing you, you can request an evaluation visit. This
homes. will give you the chance to interact with the
Depending on where you live, you may have staff in a comfortable environment and get a
multiple options when choosing a hospice better idea of how the services will work. You
provider. Just as carefully as you chose the may also want to research the reputation of
health care team to treat your mesothelioma, the agency by asking your doctor, a chaplain,
you will want to do the same in selecting a other health care professionals, and families of
hospice provider. Set up an interview and previous patients for advice and information.
asbestos Disease awareness organization asbestos > A group of minerals that take
www.asbestosdiseaseawareness.org the form of tiny fibers. Loose asbestos fib-
Provides education and advocacy on behalf of ers breathed into the lungs can cause lung
asbestos victims. cancer and malignant mesothelioma.
Malignant mesothelioma > A rare type of
Cancer Legal resource Center
cancer in which malignant cells are found in
the lining of the chest or abdomen.
cancerlegalresource.cfm Mesothelium > A membrane that covers and
Provides information on cancer-related legal protects most of the internal organs. The
issues for cancer patients and their families. mesothelium produces a lubricating fluid that
allows moving organs to glide easily against
hospice Foundation of america adjacent structures. The mesothelium has
800-854-3402 different names, depending on its location
www.hospicefoundation.org in the body.
Offers practical information about coping with pericardium > The thin layer of tissue that
death and grief, a blog, and teleconferences. forms a sac surrounding the heart.
peritoneum > The tissue that lines the ab-
Mesothelioma applied research Foundation
dominal wall and covers most of the organs
in the abdomen.
Provides education, support, and advocacy pleura > A thin layer of tissue that covers the
for mesothelioma patients. MARF also funds lungs and lines the interior wall of the chest
research and has established a grant pro- cavity.
gram to help newly diagnosed patients travel
to consult with an expert. SOURCE: National Cancer Institute
national association of hospital hospitality . . . . .
houses Content by Jennifer Klem, PhD, Nanette Lavoie-
800-542-9730 Vaughan, MSN, APN, and Lena Huang
www.nahhh.org Cover Illustration by Jan Pults
Provides accommodations for patients receiv- Published in CURE Fall 2009
ing medical treatment away from home.
InformatIon presented Is not Intended as a substItute
for the personalIzed professIonal advIce gIven by your
national hospice and palliative Care health care provIder. although great care has been taken
organization to ensure accuracy, cure medIa group and Its servants
or agents shall not be responsIble or In any way lIable
for the contInued currency of the InformatIon or for any
www.caringinfo.org errors, omIssIons, or InaccuracIes In thIs publIcatIon,
The NHPCO is an association of programs whether arIsIng from neglIgence or otherwIse or for any
that provide hospice and palliative care. consequences arIsIng therefrom. revIew and creatIon of
content Is solely the responsIbIlIty of cure medIa group.
all rIghts reserved. no part of thIs publIcatIon may be
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