Learning about Sarcoma

Dr. Gary Friedlaeder, Learning about
Sarcoma
February 13, 2011Welcome to Yale Cancer Center Answers with doctors Francine
Foss and Lynn Wilson.  I am Bruce Barber.  Dr. Foss is a
Professor of Medical Oncology and Dermatology, specializing in the
treatment of lymphomas.  Dr. Wilson is a Professor of
Therapeutic Radiology and an expert in the use of radiation to
treat lung cancers and cutaneous lymphomas.  If you would like
to join the conversation, you can contact the doctors
directly.  The address is canceranswers@yale.edu and
the phone number is 1-888-234-4YCC.  This evening, Francine
and Lynn welcome Dr. Gary Friedlander.  Dr. Friedlander is the
Wayne O. Southwick Professor of Orthopedics at Yale School of
Medicine and he joins us this evening for a conversation about
sarcoma.  Here is Francine Foss.Foss
 Could you start us off by explaining to our audience what a
sarcoma is?Friedlander
In general, cancer is referred to in two senses, one are the
carcinomas. Those are the far more common variant which include
origins in the epithelial or lining cells, or glandular
cells.  These include tumors arising in the breast, prostate,
lung, or kidney.  The sarcomas arise from the support
structures.  Some of them are in soft tissue; they may be
fatty in origin or fibrous in origin, such as muscle.  Others
are hard tissue like bone and cartilage.Wilson
How common are sarcomas and are certain types more common than
others?Friedlander
They are relatively uncommon.  My life revolves around them so
for me they seem to be relatively frequent, but in fact, they are
only 1% of all of the cancers. Of the 1.5 million cancers that
arise in the United States each year, only 1% of those are
sarcomas.  Some of them are a little bit more common than
others, and some of them are more common in children and others
more common in older ages.Wilson
What are some of the common types and are those different between
children and adults?Friedlander
Yes, children's sarcomas are very frequently Ewing sarcomas or
osteosarcomas, sarcomas that arise in bone.  In middle age and
later in life, we more commonly see cartilage related, or
chondrosarcomas, some of the fibrous sarcomas, malignant fibrous
histiocytomas and their variants are seen in middle and later
years.Foss
 Gary, you are an orthopedic oncologist, so you are basically an
orthopedic surgeon specializing in oncology.  Can you explain
what your role is in the management of these sarcomas and how you
interface with other members of the multidisciplinary team?Friedlander
It's a great question and very important.  I began my career
as an orthopedic surgeon.  I did my training in orthopedic
surgery, but I had an interest in tumors very early in my training
and at some point, I did an additional fellowship in
musculoskeletal oncology, which is a variant of surgical oncology
related to the musculoskeletal system. But I could not possibly do
what I do and certainly not as well as I am able, if it wasn't for
the team approach, and I work every day with colleagues like
yourselves, of course, from medical oncology to radiation oncology,
the radiologist, the pathologist, the entire support team is
absolutely critical to doing this correctly.3:59 into mp3 file 
http://yalecancercenter.org/podcast/feb1311-cancer-answers-friedlaender.mp3Foss             
 Can you just tell us how patients present with sarcomas?Friedlander
Sarcomas, like most other lumps and bumps are either perceived as
pain or as something growing, as a lump.  Many people notice
the pain when they bump their lump and so they frequently will go
to their primary care physician.  Most people do not like to
think they have a tumor.  When they hurt themselves or they
feel a lump or a bump, they generally relate this to some form of
injury, but when those symptoms do not go away, do not respond the
way a usual bruise, strain or sprain might, they often seek help
from their primary care physician and those diligent individuals
send them on to people like myself.Wilson
What happens at that point Gary, what sort of evaluation do you
do?  What sort of testing?  How is the diagnosis actually
made or confirmed?Friedlander
These people go through very much the same process as for any
disease or disorder.  They need to be heard.  You need to
listen to them and hear what it is they feel or perceive, what they
are concerned about and some people are concerned about cancer and
do not have any other symptoms and just need someone to listen and
examine them and examine them carefully for lumps, bumps, changes
that might signal something going on.  That leads generally to
some imaging studies, which are very helpful.  They are
remarkably good, and especially sensitive today.  It might be
a plain x-ray to begin with and depending on levels of suspicion,
we move on to other studies such as CAT scans or MRIs to help
define that there is in fact something going on.  All tumors
are not malignant, there are benign tumors and there are malignant
tumors, but once the tumor is identified, image wise, and sometimes
by feel, a biopsy often helps.  The combination of the story
they give, the physical finding they present with, and the imaging
pictures that display some characteristics, you can often get very
close to the diagnosis and understand what is going on, but often
it requires, not always but often, a biopsy.Wilson
For the biopsies of sarcomas, tell us a little bit about some of
the techniques and the important considerations, because I
understand that biopsying a sarcoma or a suspected sarcoma may
require different types of biopsy or procedures than making the
diagnosis of breast cancer, for example.Friedlander
That's correct.  The two options in biopsies are with a
needle, some novocaine, and a special needle and that can be very
very effective, especially in the hands of the well-trained
radiologist that we have here and at most major medical
centers.  It retrieves a very small piece of tissue if it is
placed in the correct orientation, so that it does not interfere
with surgery that might be required, that small piece of tissue can
often give us tremendous clues. We have pathologists who are used
to looking at very small samples and coming to very accurate
conclusions.  We have done some studies on that.  The
other option is called an open biopsy and that is done generally in
an operating room, either in a radiology suite or in an
office.  Open biopsies are done in the operating room with a
small incision.  Again, it has to be placed properly, that
incision, so it does not interfere with the ability to take the
tumor out with the least amount of impairment later on, but8:14 into mp3 file 
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that gives us a much bigger amount of tissue to work with if the
needle biopsy is insufficient or inappropriate.  Then, again,
it is a matter of looking under the microscope, and a well-trained
pathologist familiar with unusual disorders and diseases with the
benefit of multiple techniques generally can confirm the
diagnosis.Foss
 Gary, can you tell us how often, when a patient presents with a
lump in a muscle or bone, how often is it cancer and how often is
that benign? What are the benign things that could do that? 
That is a tough question, but I think an important question for
people listening.Friedlander
That is extremely important and in fact, it is one of the
highlights of my day when somebody comes in concerned about a bump
and I can tell them, yes there is a lump, but this is harmless,
this is benign, this is not going to do the things that you are
concerned with and the majority of lumps are benign.  The vast
majority of lumps are benign.  I could put a figure on it but
I must admit I am guessing, at least three quarters of the lumps
that I see turn out to be benign. They often need to be
followed.  They can weaken the bone, they can cause
impairments, they can be frustrating and they can limit activities,
but they are benign and ultimately their treatment is different
that it is for high-grade malignant tumors, and these high-grade
malignant tumors can also be handled much more successfully today
than in years past.Wilson
Do these tumors spread to other locations of the body sometimes, if
they are malignant?Friedlander
To me, one of the characteristics of a malignant tumor is its
ability to spread, to go to other parts of the body, and the tumors
that I see, the malignant sarcomas that I see, have a tendency to
go to the lung and then to other organs within the body and to
other bones, or bones in particular.  Just to be clear, people
talk about bone tumors and may be referring to one of two
situations.  One is what is termed a primary bone tumor,
something that starts in that bone, osteosarcoma being the classic,
the prototype.  Another form of, if you will, bone cancer and
far more common are tumors that came from other parts of the body,
lungs, breasts, prostate, and came to bone secondarily or are
metastatic lesions.Foss
 Another point with respect to that is we have been talking a lot
about lumps that we can kind of feel in the arms and legs and some
of these soft tissue sarcomas can occur inside the body as well and
it can be harder to pick these out.Friedlander
That is absolutely correct and people know their bodies, and often
have a sense that something is not correct and if those feelings
persist, I think they really should check with their primary care
physician.  Very often, a diligent evaluation will help them
understand that it is nothing to be concerned about, but this is
the way many cancers are found.Foss
 You also talked a little bit about high-grade sarcomas, is there a
grading system for some of these tumors?  Can you tell us a
little bit about that and what that means to you as a surgeon and
to us as a medical oncologist?12:12 into mp3 file 
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 Friedlander   Benign is on one end of the spectrum and
what is preferred to as high grade, is on the other end of the
aggressiveness spectrum with intermediate stops.  In addition
to benign, there is low grade, then intermediate grade, and high
grade.  The primary significance for me as a surgeon is how
those lumps, how those tumors are best removed.  When you want
to remove a high-grade tumor, you want to be as certain as possible
that you got every last cell out of this tumor and they do not
always have very smooth well-defined margins, so you take out the
tumor with an envelop of normal tissue.  The more aggressive
and high-grade, the thicker that envelop must be.  As tumors
get more towards the benign end of the spectrum, that envelop can
be rather thin.  So I need to know which end of the spectrum
the tumor is on.  It turns out that there are really only two
approaches to taking these tumors out, one with the very thin
envelop, one with a very thick.  It turns out that benign and
low grade can often be handled with a rather thin envelop and
intermediate and high grade need to be treated more
aggressively.Wilson
Briefly, what are some of the risk factors for sarcoma?Friedlander
It is not a brief answer.  For example, there are some
diseases like Paget's disease that older individuals get in the
bone that predispose to cancer.  There are some people that
you and I see together who have required radiation for one very
good reason or another, but that does predispose to tumors later
on, things of that nature.Wilson
We are going to take a short break for a medical minute. 
Please stay tuned to learn more information about sarcoma with Dr.
Gary Friedlander.  Wilson
Welcome back to Yale Cancer Center Answers.  This is Dr. Lynn
Wilson.  I am joined by my co-host Dr. Francine Foss. 
Today, we are joined by Dr. Gary Friedlander and we are discussing
sarcoma.  Gary, we have actually covered a fair amount of
detail on sarcoma already but let's shift15:55 into mp3 file 
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over to treatment information and discuss with our listeners some
of the different treatment options and how those are integrated
into the treatment of a patient with a sarcoma.Friedlander
This is a very exciting area.  I began my interest in tumors
about 40 years ago, and our opportunities, our success rates are so
much better today that it is truly very gratifying.  The
surgical component of treatment, in partnership with the many I
have described and will come back to later, the surgical component
first and foremost is to get rid of the tumor to remove the part
that has the disease in the soft tissue or bone.  Let me talk
about bone for a moment.  When I began, if a tumor was in a
portion of a bone, the treatment recommended almost invariably was
an amputation.  Amputation is still very important and can be
a very good operation for certain circumstances, and again, we can
come back to that, but far more often with earlier detection and
with the imaging studies that are available, it is possible to
remove the diseased part of the skeleton and replace it with
something very functional, what we call limb-sparing tumor
resection.Wilson
Could you give us an example of the type of procedure that might
fit into that category?Friedlander
For the moment let's envision a tumor that involves the bone just
above the knee, the thigh bone is called the femur and towards the
knee, may, for example, have a tumor in it. And I or my colleagues
can remove that end of the thigh bone which joins together with the
shin bone, or the tibia, to make the knee, and depending on the
size of the tumor, there may be a gap created by that removal of
several inches or a considerable number of inches and I have two
major ways to repair that gap. One is to take a bone graft that was
donated at the time of someone else's demise to a bone bank where
it is properly screened and available and I can get another portion
of the thigh bone that looks like the one I removed and put that in
place and there is a process by which the body accepts it and heals
it.  It is a lengthy process, but it works quite well. 
The other option is to make up that difference with metals and
plastics, very similar to the knee replacements, hip replacements,
shoulder replacements, elbow replacements, and ankle replacements
that we do for arthritic disease, but customized in a way that
allows really excellent, not completely normal, but very excellent
function, cemented it in place and it is able to be used within a
relatively short period of time.Foss
 Are those cadaveric bone grafts available everywhere or is that
only available in a specialized center?Friedlander
Today, most of these cadaveric or bone donated parts are retrieved
from centers around the United States, tissue banks that are
specifically set up to acquire, process, and distribute these
products and can be obtained almost anywhere.  The people who
are familiar with their implantation tend to be in academic medical
centers like our Smilow Cancer Hospital.Foss
 What about if the patient has a tumor in muscles, say a functional
muscle like the biceps in the arm, do you have to remove that
muscle? If you remove it, what can you do to improve function?20:28 into mp3 file 
http://yalecancercenter.org/podcast/feb1311-cancer-answers-friedlaender.mp3Friedlander
You have to go back to job #1, get rid of all the tumors regardless
of what that means in terms of function.  If you start to take
the tumor out and spent too much time thinking about the function,
you might cut corners that leave tumor.  It does not mean you
have to take things unnecessarily and that's where skill and
experience comes in and it does not mean you have to take out the
entire muscle. Fortunately, there is a great deal of redundancy in
the body and often other muscles can help compensate for getting
rid of a nasty problem.  Under some circumstances, you can
rearrange muscles, you can take a muscles that does one thing and
reattach it in a way that it does something that is more
important.Wilson
How has the prognosis changed, Gary, over the last decade for
sarcoma? Obviously it has to do with treatment advances, surgical
advances, multidisciplinary care, but discuss with our listeners
how things have changed?Friedlander
When I first became interested, and I have already admitted it was
about 40 years ago, the likelihood of surviving osteosarcoma, bone
cancer, primary bone cancer, was about 10% at two years. 
Today, it is probably 70% to 80% of people that have osteosarcoma
that are cured.Wilson
 That is impressive. Friedlander
It is not all my fault.  I would like to think I can
contribute to that but people like Francine and my medical oncology
colleagues, and my radiation therapy colleagues, have really made
the difference.  Multidrug chemotherapy has been absolutely
spectacular and the imaging studies we have for early detection
both of the primary tumor, of metastatic disease, enormous
differences today.Foss
 Are you alluding to the role of PET scan to detect metastatic
disease here?Friedlander
PET scan is one of the very very superb ways for looking for
metastatic disease, many of the sarcomas that I deal with
metastasize first, very often, to the lung. CT scans have been
around for a while, but they can be extremely helpful in
surveillance after tumors have been removed.  CAT scans and
occasionally MRIs.  We use MRIs very frequently here at the
site of the original tumor to be certain that it is not recurring
and the recurrence rates now are down in the 1% and 2% range, if
that much.Foss
 Can you talk a little bit about the role of radiation therapy and
whether you would, in some instances, radiate before you do a
surgical procedure? Friedlander
This is choosing between two correct options. My colleague Dieter
Lindskog, trained at an institution for his tumor experience that
preferred preoperative radiation.  In my training in Boston,
in that regard, tended to use radiation after the resection and
there are some good reasons one might do it before or after,
routinely, and there are some specific reasons why you should do
it
                     24:12 into mp3 file 
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before or after, but that's why I will call Lynn and his colleagues
and say, what do you think?Wilson
There is obviously a lot of variability depending on the location
and the size and how it will affect your procedure, and it can be a
complex decision which gets back again to the multidisciplinary
aspect of things and how critical it is for all of us to work
together with specialists taking care of these patients.Friedlander
We meet regularly for this particular purpose to make sure that we
have that kind of communication.  We get together as medical
oncologist, radiation oncologist, surgical oncologist, pathologist,
and radiologist, on a routine basis and go over cases even though
we know what we are likely to say and what each other are likely to
determine, but that keeps that face-to-face communication very much
in our minds, and as you know, when you are clustered together in a
superb environment like Smilow, it is just a matter or turning
around and finding the right person or picking up the phone and
clarifying some of these joint decisions that need to be made
together.Foss
 Do most of your patients end up seeing the medical oncologist
prior to their surgery or does it usually happen after the surgical
procedure? Friedlander
If at all possible, I very much prefer them to meet the other
members of the team prior to surgery.  There are times when
the surgery must proceed rather quickly, but for the prototype
sarcoma that I was referring to before, very often we consider
preoperative chemotherapy.  This is a little bit more
difficult to explain to the patient because intuitively they come
to a surgeon and want to cut it out, they want it removed. 
The first thing is they just want to get rid of it, they want it
out, even though they are going to need chemotherapy.  Well,
it is a one-two punch, and whether you hit them first with your
right or your left hand, can make a little bit of a difference
because what is extremely important is to make sure the tumor does
not spread, and the best way to do that is to start the
chemotherapy.  Surgery has no effect on spread other than
taking away the primary, and when I see patients there may already
be cells in the body and I am depending on you to make sure our
chemotherapeutic agents hunt those down and kills those
cells.  We can also use other medicines that make it hard for
those cells to implant and grow in other parts of the skeleton.Foss
 So most patients with high-grade sarcomas will get chemotherapy
either before or after?Friedlander
Most of those patients where we have good evidence, and we often
do, the chemotherapy makes a difference, get that form of
treatment, and often get at least part of it before surgery.Foss
 Could we just touch on the issue of surgery in a patient who
already has metastatic disease?  If you see somebody, you make
the diagnosis, and then you do your scans and you find out that
there are already metastases, do you still try to approach the
primary tumor by resecting it?Friedlander
This has to be individualized, but that brings up another very
exciting opportunity and where metastatic disease used to be
considered irretrievable, if you will, that's clearly not the case
any27:52 into mp3 file 
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more.  For example, metastatic disease to the lung often can
be cured by resection and it may require even more than one
resection, but it is not a zero game at this point, probably at
least 20% of people with metastasis to the lungs, if they are
relatively few, can be turned around, and in conjunction with
chemotherapy.  The other thing tumors do to bones is weaken
them and we are very sensitive to wanting people to enjoy their
life opportunities, and we have to be thoughtful, sympathetic,
compassionate, and know when to operate and when not to
operate.Wilson
What sort of rehabilitation is associated with this?  I know
it is a difficult answer to give depending on what part of the body
gets an operation, but I would suspect most of your patients need
to go through a rehabilitation program, which could be fairly
lengthy for them.Friedlander
They do.  For the metallic implants, the rehabilitation gets
started quicker and probably does not need to last quite as
long.  Many of these can be home exercises.  For the bone
grafts, the donated bone, they need a lot more protected time as
biology heals.Dr. Gary Friedlander is the Wayne O. Southwick Professor of
Orthopedics at Yale School of Medicine.  If you have
questions or would like to share your comments, visit 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 on the Connecticut Public
Broadcasting Network.