Understanding Sarcoma

Dr. Gary Friedlaender, Understanding
Sarcoma
 May 24, 2009Welcome to Yale Cancer Center Answers with Dr. Ed Chu and
Francine Foss, I am Bruce Barber.  Dr. Chu is Deputy Director
and Chief of Medical Oncology at Yale Cancer Center and he is an
internationally recognized expert on colorectal cancer.  Dr.
Foss is a Professor of Medical Oncology and Dermatology and she is
an expert in the treatment of lymphomas.  If you would like to
join the discussion, you can contact the doctors directly. 
The address is canceranswers@yale.edu and
the phone number is 1888-234-4YCC.  This evening Ed and
Francine welcome Dr. Gary Friedlaender.  Dr. Friedlaender is
the Wayne O. Southwick Professor of Orthopedics at Yale School of
Medicine and an expert in the treatment of sarcoma.Chu
 Why don't we start off by discussing what sarcoma is because I
suspect many people out there listening may not have a real good
sense of what type of cancer that refers to.Friedlaender
It is a type of cancer, and cancers in general are broken down into
two broad categories.  One are the carcinomas, malignances of
epithelial cells, surfaces of organs and organ systems in the skin,
but what we are talking about are sarcomas, cancers of support
tissues, such as connective tissues, bone, cartilage, muscle and
some of the tissues that are in the support organs or tissues like
blood vessels and nerves.Chu
 There is also soft tissue sarcomas and non-soft tissue sarcomas,
how do you break down those two groupings?Friedlaender
We usually refer to bone and softer tissues, cartilage gets caught
in between a little, but there is bone on one end of that spectrum
and then there are tissues like fat and muscle, the softer tissues,
on the other end, and we call those soft tissue sarcomas.Foss
Gary, can you tell us a little bit about how common these types of
sarcomas are?Friedlaender
I'd be glad to Francine.  They are uncommon in general, about
1% of the overall cancer population.  For me, it is something
that I deal with everyday, and that's what happens when you become
a sub-specialist and part of a team like we have here at
Yale.  These are things that I see all the time, but really
are only about 1% of the pool of cancers in general.Foss
What ages do they occur in? Are they common in children, and do
they occur in older adults as well?Friedlaender
That's an important question.  They are tumors that occur at
all ages, but the specific kind of sarcoma one finds in children is
different than the kinds of sarcomas of older individuals. As an
example, retinoblastomas are more common in very young
children.3:12 into mp3 file 
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 Ewing sarcoma and osteosarcomas occurs in later childhood, young
adulthood, and then we see fibrosarcomas and chondrosarcomas that
more commonly appear in older age.Chu
 And there are also sarcomas that can involve the GI tract as well,
is that correct?Friedlaender
I am an orthopod, an orthopedic surgeon, so I am aware of that, but
I don't take care of them as much, but indeed they are the most
common sarcomas.  There are less than 10,000 sarcomas
occurring each year in the United States, of which a third are GI
tract.Foss
We talk about low grade and high grade sarcomas, I wonder if you
could tell us if there are sarcomas that are not truly malignant
tumors? Is there a spectrum in terms of the behaviors of these
tumors?Friedlaender
It's a very important question and the answer is yes, there is a
spectrum.  The spectrum starts on one end with benign, and on
the other end with what we term high grade.  There are
intermediate stops between those two extremes that are generally
termed low grade and intermediate grade.  Treatment, on the
other hand, tends to be either focused on benign and low grade or
on the higher grades, and so when you find tumors in between,
intermediate, you have to choose whether to, if you will,
over-treat or under-treat and by convention we over-treat, if you
will, we lump them with the high grade.  And the treatment is
different for benign and a low-grade cancer, and intermediate or
high-grade cancer.Foss
It seems funny to use the terms benign and tumors, or benign and
cancer in the same breath.  These benign sarcomas or lesions,
are they really tumors, or are they cancer?Friedlaender
Let me back up for a moment.  The word tumor to me represents
a lump, and you can have a benign tumor or a malignant tumor. 
In common parlance, when you begin to use the word tumor, it often
conjures up the notion of something nasty, and it's important that
as we talk to each other and as we talk to patients, to define the
terms. I find myself doing this regularly with patients, and almost
apologizing sometimes for using the word tumor, when I mean,
potentially benign.  Your point is well taken, and we should
start out with the definitions.  If I use the word tumor, I
should let you know if I am talking about a benign tumor or a
malignant tumor.  There are no benign malignant tumors; that's
incongruence. What makes a tumor malignant is its ability to
metastasize, and once it gains that potential it is a
malignancy.  That potential may be low or high, and
consequently, our classification of low grade malignancies, but
still able to metastasize, versus high-grade malignancies.  We
are sometimes caught in the dilemma of conventions that have
classified a tumor as a low-grade malignancy, despite its inability
to metastasize, so the terms get fuzzy6:56 into mp3 file 
http://www.yalecancercenter.org/podcast/Answers_May-24-09.mp3sometimes. Trying to keep things relatively simplistic,
malignant means it can spread, benign means it can't.Chu
 Gary, what do we know about the risk factors that are commonly
associated with the development of neurosarcoma? Is there any
genetic component to the risk factors?Friedlaender
Absolutely, and if I were to point to an area of progress it's our
understanding of why these tumors happen under a certain
circumstances. It used to be very easy to say, "I don't know, we
just haven't figured it out yet," and for a large number of tumors
we don't know, but we do have increasing information about risk
factors.  For example, radiation, something we use to treat
tumors, can, at higher doses, cause new tumors to happen years
later.  We know that certain chemicals, chemicals in our work
place and chemicals in our homes can cause cancers.  Certain
pesticides, certain herbicides, certain chemicals like arsenic that
are used in industry and in preparing fabrics; we know now can
cause cancer.  Chronic lymphedema, a circumstance that happens
when we interrupt the lymph drainage, a classic example being in
mastectomies, seems to lend itself towards the formation of some
cancers.  We know there are individuals, families, that
genetically seem predisposed to have multiple malignancies, so
there is a genetic or hereditary component.  There are
malignancies associated with HIV infection, Kaposi sarcomas. 
It's not a simple answer, but what we are finding is there are
multiple pathways to get to the same endpoint of cancer.Foss
There are a lot of patients out there now that have been treated
with radiation, patient's with prostate cancer, breast cancer, and
Hodgkin's disease for instance, that are long term survivors. To
what degree do these people have to worry about sarcomas, and how
frequently do sarcomas occur in patient's who are long term
survivors of radiation, therapeutic radiation?Friedlaender
It's a small but important number, and not cause for great alarm in
individuals. What it does tell me, these small numbers, is that
people just need to remain vigilant, they need to remain under the
care of people that can help monitor whether or not one of these
late sarcomas is developing.Chu
 Are there any symptoms that you would typically look for that
might suggest that a lump or bump in the arms or legs might be
something more serious?Friedlaender
Yes, the symptoms of a sarcoma are pain, a lump, or both.
Malignancies tend to grow and they tend to be deeper in the body
rather than just underneath the skin.  The most common lump is
a harmless fatty tumor underneath the skin, and if it's less than 2
inches it almost10:47 into mp3 file 
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 never is malignant.  I have given up saying always and never,
but there are some guidelines, and a lump that is growing deserves
some additional attention and a healthcare professional should be
able to guide you as to whether further studies are important to
clarify what's going on.Foss
When we are talking about imaging studies, we have PET scans, MRIs
and CAT scans, can you tell us a little bit about how each one of
those is used in the diagnosis and follow-up of patient's with
sarcomas?Friedlaender
Yes Francine, and I would like to just back up a half step, because
those are part of the tools that their physician is going to use to
help clarify what is going on, beginning with a good discussion,
history, and a physical examination, which often can clarify
whether or not something is in need of more evaluation.  Then
you get into plain x-rays, which are very helpful for bone, and are
not as helpful for things that happen in the soft tissues. 
CAT scans and MRIs are extremely valuable imaging opportunities
that help us see the inside of the body very clearly and see the
soft tissues.  CT scans see bone very well and MRI does not
see bone at all, but it sees the things around bone, so they are
complementary.  An MRI also helps us know a little bit about
the biology more than a CAT scan.  An MRI does not involve any
radiation while a CAT scan is based on radiation.  With an MRI
you put yourself in a tube that is surrounded by a magnet, that
magnet gets turned on and makes a huge amount of racket, but what
it is doing is pulling the electrons in each molecule a little bit,
they turn off that magnet and everything goes back where it
belongs, but sends out a little blip of energy that the computer
then picks up in three dimension.  We then ask the computer to
pretend we are looking at a person from the front, from the side,
or literally making slices through them and by assigning some
arbitrary colors to different tissues types, we can sort out both
anatomy and biology or physiology; very-very powerful.  PET
scans are also very special, along with bone scans, and there are a
number of tools that are worth mentioning, but the PET scan in
particular helps us look at the metabolism of individual cells and
the metabolism of cancer is often different than the metabolism of
normal cells.  It is a study that looks at us from head to
toe.  An MRI is a study that has to be focused on a small
area.Chu
 If there is a suspicious lesion that's identified on either CT
scan or MRI, what would be the next step in terms of the workup
process?Friedlaender
Putting all the information together is very important, but
ultimately the decision or the answer often will come from a
biopsy.  Deciding when to do a biopsy and how to do a biopsy
is critical, and that's part of a team decision very often, but the
surgeon and the radiologist play the central roles.  For
example, there are needle biopsies where with some14:33 into mp3 file 
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 Novocain and a very special needle you can get a small piece of a
lump or bump, even if it's deep within the body, using a CAT scan
machine to guide you or an ultrasound machine to specifically
target even a small lump.  The advantage is it's not an
operation, the disadvantage is it gets a very small amount of
tissue and you need a very skilled pathologist, the people who look
at these samples, to help interpret them.  About 90% of the
time a needle biopsy is sufficient.  The other alternative is
termed, an open biopsy, where you are in the operating room more
often than not, although they do it in some offices, but you make a
modest incision and take a representative portion of the lump to
look at under the microscope and that generally gives us the answer
in 99 point something percent of the time.Foss
Thank you Gary, we would like to talk more about the treatment for
sarcomas when we come back after this break.  You are
listening to Yale Cancer Center Answers and we are here today with
Dr. Gary Friedlaender.Foss
Welcome back to Yale Cancer Center Answers.  This is Dr.
Francine Foss and I am here with Dr. Ed Chu and Dr. Gary
Friedlaender discussing sarcomas.  Gary, we talked a little
bit about the diagnosis of sarcoma, can you tell us what happens
next after you have a definitive diagnosis?Friedlaender
Yes, and I can't help but reminisce a little bit.  When I
first became involved with bone tumors as a resident, and you can't
see me on the radio but that was a while ago, the success rate was
rather oblique, and indeed for an osteosarcoma, the survival rate
for two years was about 10% to 15%; very miserable.  Today,
the survival rate, the cure rate, not just the two years survival
rate, but the cure rate, is around 80%, and the difference is a
reflection of the17:35 into mp3 file 
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 team that gets pulled together to take care of these individuals
and the tools that each of those members has to make a difference.
Some parts of those are diagnostic with the radiologist and
pathologist, some of them are interventional by surgeons, sometimes
radiation and often chemotherapy.Foss
Do all patients require an amputation?Friedlaender
Not at all, and in fact, one of the very gratifying changes over
these years has been our opportunities as surgeons,
parenthetically, because you as chemotherapists have been able to
deal with systemic disease better, but we can now isolate the part
of the body that has a tumor, be that bone or muscle, and remove
just the part that has the tumor and then use some very creative
opportunities to reconstruct the skeleton.Chu
 At Yale you lead the multidisciplinary team that focuses on
sarcoma. Can you tell us a little bit more about the specific
players who make-up that team, that team-based approach?Friedlaender
Absolutely, and with our new Smilow Center Hospital and our
additional leadership this is a very exciting time to bring these
people together in a more formal way and perhaps even a bit more
efficient. I can tell you that as of right now, it is superb, and
it involves a patient seeking out help, sometimes me as a surgeon,
sometimes you as a medical oncologist, but most often going to
their primary care physician and saying it hurts, I feel a lump,
and then they get referred into our system. It's each of our jobs
to call the team together, depending on what we think the patient's
needs might be.  Early on, again the radiologist is absolutely
critical, the radiologist I work with sits 15 feet away from me
while I am seeing patients and the partnership is enormously
important and effective.  They help me interpret what I see,
help me decide what additional tests to get. Then a biopsy is done
if necessary, and the pathologist becomes the center of attention
and it is always important to know what you are dealing with,
because that determines what the alternatives are and there are
always choices.  That's when I as a surgeon, you two as
medical oncologists, and our colleagues in radiation therapy get
together as we do formally and informally. We see each other in the
hallways, we see each other in conferences, whatever it takes to
make sure this patient has the benefit of all those opinions, and
we come to a consensus and the consensus may include, again,
surgery, radiation, chemotherapy, one of them, all of them,
sequencing those. There are also all of the other support people
that go along with the social aspects or the psychological aspects
of people that have these disorders, and the pain management that
they deserve.  They rehabilitate people by body, by mind,
occupational therapists, physical therapists, psychologists, pain
management specialists, all are part of the team.21:23into mp3 file 
http://www.yalecancercenter.org/podcast/Answers_May-24-09.mp3Foss
Can you talk about some of the advances with respect to helping you
rebuild say a bone or muscle or tissue that you have to excise?Friedlaender
Certainly, let me focus on bone for a moment, and picture if you
will that the tumor is in the lower half of the thigh bone, just
above the knee.  The first step is to remove the lower half of
the thigh bone or femur, and then there are choices about how to
reconstruct it and they fall into two general categories.  One
is a bone from another person, a donor, someone who has passed
away, some generous person or family. Just as we have the ability
to transplant hearts, lungs, livers, kidneys, and corneas, we also
have the availability of large segments of the skeleton in a
similar way to replace a similar part of our anatomy.  And
there are some advantages and disadvantages to that approach. 
The other option is the metals and plastics that we are so familiar
with joint replacements, total hip replacements, and total knee
replacements, that have allowed us to build upon those concepts and
replace large portions of the skeleton with metal and plastic.Chu
 Are there are any long-term complications with using these metal
prosthesis or joint replacements?Friedlaender
There are sometimes issues in the short run, but most commonly in
the longer run. In that you have to bind them or bond them to the
skeleton.  You have to insert a stem or some other mechanism
to bind these, often by bone cement, to the skeleton, and over a
long period of time that bond can deteriorate.  In a regular
total hip replacement or total knee replacement, we think in terms
of 15, 20, 25 years of good outcome, excellent bond, no need to
change. In these larger, we call them megaprosthesis, that bond
also breaks down overtime, but probably doesn't last quite as
long.  The good news, if you will, is that when these bonds
wear, they can be replaced. And I can tell you that 2009 model has
been better than the 1999 model and I have every reason to believe
in the future we are going to get better and better.Chu
 Is there any way in terms of imaging or some other kind of test
where you can figure out who is going to have trouble with the
bonds breaking down, or is it just a matter of the patient comes
back for followup and says there is pain, discomfort, and just
can't do the same kinds of things they were able to do
previously?Friedlaender
These patient's become very important to us as physicians, as you
know, and we windup seeing each other over a very long period of
time, a part of the practice I enjoy.  Most of these patient's
we can help dramatically and we get to know them as they go through
life, but periodically, its our responsibility to make sure they
are healthy and they come back for their 1000-mile checkups. Part
of that evaluation includes making sure that their prosthetic25:14 into mp3 file 
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 device is in good shape and it's a combination of x-ray, plain
x-ray generally, and symptoms, that is pain, that allows us to
determine how healthy that bond is and if it deteriorates you come
to mutual decisions about how to address it, which is usually to
replace it.  And that starts the clock all over again, so they
can enjoy school, play, work, and enjoy their life as usual.Foss
We talked a little bit about the use of chemotherapy in the
management of sarcomas and I wonder if you could comment on some
more investigational approaches such as isolated limb perfusion or
hyperthermia, and whether these actually play a role nowadays in
our treatment algorithm for patients?Friedlaender
Yes, thank you for the question.  In terms of context, cancer
has two parts to it.  One is where it begins, we call that the
primary tumor.  As the surgeon, I and the radiation therapist
usually have responsibility for the primary, but what makes cancer
dangerous as we talked about earlier, is its ability to spread, and
until we found ways to deal with the rest of the body and the
potential spread through chemotherapy, and in particular multidrug
chemotherapy, we didn't make the dramatic success rates that we
enjoy today and set the stage for even better tomorrow.  Now,
the way we give the chemotherapy can be targeted at the primary
tumor as well, and we can deliver some of these drugs directly into
the tumor, the primary tumor, by inducing or putting the catheter
into a blood vessel that feeds them; limb perfusion
techniques.  And we find in certain types of tumors it has
been extremely helpful.Chu
 Any new developments from your program in terms of bone research?
I know that's been an area of your specific interest.Friedlaender
Absolutely, we have found in our parallel lives, thinking about the
skeleton, many ways to control the way bone repair itself, bone
regenerates.  To me its marvelous, whether you are just
sitting there, have fractured your bone, or have a bone graft,
there is a process of cells that get together and replace old bone
with new bone. We now have isolated many of the molecules that turn
on the process of making new bone and this I believe is going to
play a very important role in improving the reconstructions that we
are already doing.Chu
 We look forward to hearing more about what's going on in that area
of research from you on a future show.  You have been
listening to Yale Cancer Center Answers and we would like to thank
our guest expert for this evening's show, Dr. Gary Friedlaender,
for joining us.  Until next time, I am Ed Chu from the Yale
Cancer Center wishing you a safe and healthy week.28:36 into mp3 file 
http://www.yalecancercenter.org/podcast/Answers_May-24-09.mp3If you have questions or would like to share your comments, go
to yalecancercenter.org where you can also subscribe to our podcast
and find written transcripts of past programs.  I am Bruce
Barber and you are listening to the WNPR Health Forum from
Connecticut Public Radio.