Cancer Answers: Rare Skin Malignancies, January 30, 2011

Dr. David Leffell, Rare Skin Malignancies
January 30, 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, Lynn
welcomes Dr. David Leffell to the program.  Dr. Leffell is the
David Paige Smith Professor of Dermatology and Surgery and Deputy
Dean for Clinical Affairs at Yale School of Medicine.  Here is
Lynn Wilson.Wilson
Let us start off by having you describe what skin cancer is.Leffell
Skin cancer represents a broad category of malignant or cancerous
tumors or growths of the skin.  I think that many listeners
are most familiar with the terms basal cell cancer and squamous
cell cancer, and that is largely because these are the most common
cancers that we encounter. In fact, basal cell cancer itself is the
most common cancer in humans, but in addition to those two cancers
commonly seen in the skin, melanoma, malignant melanoma, is also
considered a skin cancer.  It is a malignancy or a cancer of
the pigment cells of the skin, and it also is unfortunately more
common than we would like.Wilson
In what age groups do we typically see basal or squamous cell
carcinomas?Leffell
Lynn, that is a great question.  It used to be that basal cell
cancer and squamous cell cancer was seen in individuals in their
50s, 60s and later, but we are seeing patients with basal cell
cancer at a much younger age and, in fact, we have a research study
going on now to try to understand why basal cell cancer is
developing in individuals under 40.Wilson
What are some of the lesser known types of cancers?Leffell
It is interesting, the human body is an amazing piece of machinery
and it is made up, of course, hundreds of thousands of complex
parts, organs, and organs are made up of cells, and cells even have
little structures within them that help us function
biologically.  But the opposite side of that coin is that each
of these cells potentially could go sour. In some ways that is what
cancer is.  When we talk about the skin, the skin is a complex
organ, believe it or not, and we like to think that it is the
largest organ in the body and it includes many types of
cells.  It includes the epidermal cells, or the top-layer
cells, and from the epidermis basal cell cancer and squamous cell
cancer develop.  I already mentioned that malignant melanoma
can develop from the pigment cells that are also found in the top
layer of the skin, but beneath the epidermis, which in reality is
only about the thickness of a sheet of paper, is the dermis, the
second layer of the skin, and there are many different cells types
there, and here is the rub, some of those cell types can at times
develop into cancer, not very often, and in fact as a group they
represent rare skin cancers, and these include cancers or
malignancies of the skin that are called, and get ready because
these are long names and we in medicine actually tend to abbreviate
them which maybe we will do for purposes of this discussion, but
the first one is an atypical fibroxanthoma, abbreviated as AFX,3:55 into mp3 file 
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dermatofibrosarcoma protuberans, abbreviated as DFSP, and Merkel
cell carcinoma, which fortunately is brief enough that it does not
need an abbreviation.Wilson
Is Merkel cell carcinoma, or any of these other problems, are they
related to sun exposure or viral exposures?  What sort of risk
factors do we know about?Leffell
Let's talk a bit about Merkel cell carcinoma.  It is a very
rare cancer.  There are only about a thousand cases a year in
the United States, and it occurs in men generally over 60 on
sun-exposed areas, just like basal cell cancer and squamous cell
cancer, but what is very interesting and reflects our developing
knowledge and the benefits of all the investment that we as a
society are making into biomedical research, is that with Merkel
cell carcinoma, as we now know, there is a virus called the Merkel
cell polyomavirus that probably plays an important role in causing
this particular cancer.  The virus itself is not the whole
story, because we know that the malignancy, the cancer, develops
mainly on sun-exposed areas.  So clearly, the story of
ultraviolet radiation having a negative impact on the skin and
setting the stage perhaps even stimulating cancer in the case of
Merkel cell carcinoma has to be considered.Wilson
Do you feel that if someone lives long enough, that it is highly
likely that they may get a skin cancer, a basal cell carcinoma, for
example, since there are so many cases of this disorder?Leffell
Clearly the cause of basal cell cancer and squamous cell cancer is
related in many ways to the cumulative exposure to sunlight,
ultraviolet radiation.  We have not talked really about the
risk factors for skin cancer, but now might be a good time. 
The people that are most at risk for developing skin cancer are
those with light complexion, those with fair hair, blue, green, or
gray eyes, certainly those people who have a family history of skin
cancer.  I should point out that many people think that if
they have a darker complexion, they are immune to skin cancer, and
I have got to tell you that here at Yale where we see patients from
all over the state, there are a fair number of people now
developing skin cancer who do not fit the typical risk profile, but
who have had extensive sun exposure throughout their life, which of
course, is the one common factor in patients that develop most of
the squamous cell cancers and basal cell cancers.Wilson
Can these cancers spread to other parts of the body, or to lymph
nodes?Leffell
Basal cell cancer typically does not.  It can be treated
readily by your physician, and although there is typically a 40%
chance of getting another one within five years after your first,
it is very manageable.  Squamous cell cancer is thought of by
the general public as more serious than basal cell cancer, and in
some ways that is true, but in the majority of instances, squamous
cell cancer is diagnosed at an early, very treatable stage, just
like basal cell cancer. Having said that, in certain rare
circumstances, squamous cell cancer of the skin is not trivial and
it can potentially spread to the lymph nodes, and from the lymph
nodes to the lung and other areas, but in terms of Merkel cell
carcinoma, it is a serious cancer, and let me talk a bit about
it.  First of all, how do you know that you have one? 
Well, the problem with Merkel cell carcinoma is that it comes up
often like just a7:47 into mp3 file 
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red bump and it is very hard to distinguish it from a pimple early
on, or a basal cell cancer or another type of skin lump, bump, or
growth, and so it is very important that if you notice a new lump
or bump that you get it checked out by your dermatologist as
promptly as possible.  Having said that, Merkel cell carcinoma
in some ways can behave like melanoma, like malignant melanoma, and
it can travel to other organs, first, of course, to the lymph
nodes.  Part of the treatment, once the diagnosis has been
made, or in the process of diagnosis and treatment, includes
removal of the skin cancer and also an evaluation of the lymph
nodes through a technique called sentinel lymph node biopsy. 
This is a technique that allows your doctor to evaluate whether the
lymph nodes are involved by the cancer.  The good news about
Merkel cell carcinoma is that if you get it, and it is treated and
you do not develop a recurrence or spread of the cancer to other
parts of the body for three to five years, the odds of developing
those problems after that time period are extremely low. 
Generally we think of three years to five years as a cure period
for a Merkel cell carcinoma.Wilson
David, tell us a little bit about Smilow and the Yale University
skin cancer treatment history.  You are, obviously, regarded
as an international expert in these diseases.Leffell
I am most familiar with the program at Yale.  I was actually
recruited back in 1988 to start the first skin cancer, or cutaneous
oncology program, and we have been able to grow substantially since
that time and we have a very comprehensive program that includes
clinical care where we put a great focus on patient service and
quality as well as clinical research where we are actually
leveraging all of the clinical information that we develop to
advance science and our understanding of skin cancer.  Our
team helped discover the skin cancer gene in 1996, which has had
many implications for our understanding of other cancers as
well.  The other component of the skin cancer program at Yale
Cancer Center and Smilow Cancer Hospital, is our melanoma program,
and malignant melanoma, as a disease is increasing and is
potentially lethal and is of special concern in Connecticut. 
I am delighted to mention that we just recently recruited a
national leader in melanoma surgery, Dr. Mark Faries who is playing
a central role in our melanoma program. He is a surgical oncologist
and brings with him, from the John Wayne Cancer Center, many
advanced techniques and extensive expertise in the management of
malignant melanoma.  I would say that we run the gamut. 
For example, the rare cancers that we began to discuss earlier in
the show, Merkel cell carcinoma, AFX, and DFSP are treated at the
cutaneous oncology program in the dermatology department, but
frankly, we collaborate extensively in a multidisciplinary program
with the other experts at Smilow.  For example, one of the key
treatments for Merkel cell carcinoma after surgical excision is
radiation, and that is well established, and we collaborate very
closely with our colleagues in therapeutic radiology, not just with
Merkel cell carcinoma but, as you know Lynn because we worked
together on some of these projects, with complex cases of squamous
cell carcinoma for which there is not broad expertise elsewhere,
and that is one of the challenges.  I actually wish sometimes
that there were others that were more expert than we are, because
these cases can be very challenging, and in the case of extensive
skin cancers, such as DFSP and even basal cell cancer, we have the
ability to collaborate very closely with12:05 into mp3 file 
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reconstructive plastic surgeons at Yale who are also part of Yale
Cancer Center and Smilow Cancer Hospital.  While most of the
cases of skin cancer are easily treated in our unit, we are able to
at any time pick up the phone and usually have a patient seen by
one of our colleague consultants the same day, which as you can
imagine is a great satisfier.Wilson
Absolutely!  David, you are a dermatologic surgeon, tell our
listeners a bit about that, your training background, and what is
Mohs surgery?Leffell
Titles and labels are very complicated at times but they actually
represent, in most cases, a wide range of extensive training. 
Dermatologic surgery is a subspecialty of dermatology, the same way
cardiology is a subspecialty of internal medicine, and individuals
first have to finish training in dermatology and become board
certified in that specialty.  After that they pursue a
fellowship, a full year of training, specifically in skin cancer
surgery and reconstruction, and we at Yale actually sponsor such a
fellowship.  The fellowship is centered on a technique called
Mohs surgery.  Mohs surgery is actually named after Frederic
Mohs who was a surgeon at the University of Wisconsin who developed
a technique to remove skin cancer in a layered fashion, and the
advantage of that is that the cancer is removed in as conservative
a fashion as possible.Wilson
And how many Mohs surgeons or dermatologic surgeons do we have here
at Yale?Leffell
I am pleased to say that our program now has four dermatologic
surgeons.  Our most recent recruit, Allison Hanlon from
Vanderbilt University is an MD, PhD who in addition to performing
Mohs surgery and handling a wide variety of skin cancer patients,
is developing an active research program in skin cancer.Wilson
Terrific!  We are going to take a short break for a medical
minute.  Please stay tuned to learn more information about
skin malignancies with Dr. David Leffell.15:19 into mp3 file 
http://yalecancercenter.org/podcast/jan3011-cancer-answers-leffell.mp3Wilson
Welcome back to Yale Cancer Center Answers.  This is Dr. Lynn
Wilson.  Today, we are joined by Dr. David Leffell, and we are
discussing skin malignancies.  Let's return our attention to
some of the more unusual malignancies of the skin that you have
mentioned already, but let us get into a little bit more detail
about them, how they present, what sort of physicians should be
involved in their treatment and how they should be managed?Leffell
I think we can start off by talking about the atypical
fibroxanthoma, the AFX, and once you get to the level of rare and
infrequent cancers, you really butt up against the diagnostic
criteria, how do you know what kind of cancer it is and can you be
sure and what is the right treatment? Ultimately, the diagnosis of
skin cancer is made by a biopsy, and a biopsy is a very simple
office procedure where the skin is numbed with a little bit of
lidocaine, similar to what a dentist might use, and the specimen is
either shaved off or punched out of the skin and sent off to a skin
pathology lab, and I want to emphasize this, it is very important
to make sure that your skin biopsy is read by a certified
dermatopathologist or skin pathologist, especially when we are
talking about these rare malignancies, which frankly many
pathologists do not have a chance to see that often.  You
really want to make sure that if the dermatologist is concerned
that it may be one of these rare cancers that it is read by
individuals, frankly, at a university center.Wilson
Talk to us about the management of these various, more unusual
disorders?Leffell
Again these are skin cancers that develop in older people, elderly
men and most often on the scalp, and that is corroborated by the
literature, and they come up in a relatively nondescript
fashion.  They can look like a red bump or a crusted bump and
may not even alert the patient that there is something wrong. 
Let us assume that you are seeing your dermatologist on a regular
basis because you are at risk for skin cancer and the diagnosis is
made by biopsy.  The treatment for AFX is the Mohs surgery, it
is the treatment of choice because you are able to remove the skin
cancer in the office setting layer by layer, and once it is all
complete, again, in the office setting, if the wound is such that
you can perform the reconstruction then and there, it is all taken
care of at once.  AFX has a relatively low potential for
spreading to the lymph nodes and into the blood stream. 
Although in my experience, I have seen it happen.  When it
occurs, one really has to wonder whether the diagnosis was an AFX
or a cousin of AFX, which occurs less often on the scalp but does
have a potential to metastasize, but we are talking now about very
arcane issues but it is important for the listening public to know
that we, as physicians, do live in a world where we need to be
alert to many of the nuances and complexities of things that are
not routine. Once AFX is treated by the Mohs technique and the area
is repaired, or skin grafted or whatever is required, the risk of
recurrence, at least with the Mohs technique, is very low; maybe 1%
or 2%, and the patient can be reasonably sure that they are not
going to get into trouble from that.  On the other hand,
individuals with AFX are also at risk for the more common skin
cancers that we talked about at the top of the show, squamous cell
cancer and basal cell cancer, for example.  So AFX is
relatively simple, the key thing there is making sure that the
diagnosis is correct and that the treatment has been thorough and
complete.  Another entity, which I mentioned briefly but is
also a mouthful, is dermatofibrosarcoma protuberans, which we like
to refer to as DFSP for purposes of19:41 into mp3 file 
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simplicity.  This is not a sun-related skin cancer, and it is
a bit tricky because it develops very slowly, actually, in many
cases over many years so slowly, in fact, that people may not even
be aware that they have it, and it is a malignancy of fibrous
tissue, which presumably originates in the second layer of the
skin, again, that I described in the first half of the show. 
The treatment for DFSP is excision.  How do you know you might
have one? Well, they can look like anything and I do not say that
to induce a level of broad paranoia, but rather to make people
realize that if they have an area that they thought was a scar or
something firm on their arm, on their chest, really it can occur
anywhere and you do not have a good explanation for it, this should
probably be checked out because DFSP is often slightly raised,
maybe a little purple in color, a little red in color, sometimes
even brownish or tan in color.  Sometimes, you can put your
fingers around it and pinch it and actually grasp it if it is of
sufficient size, and it feels when you do that, like it is embedded
within the second layer of the skin, within the depth of the skin
itself.  The good news is, it almost never spreads to the rest
of the body and it is, again, easily treated once the diagnosis is
made, with excision, either conventional surgical excision with an
appropriate margin of safety or with the Mohs technique where again
you are studying the margins immediately and tracing out the cancer
in all of its directions until you are certain that it has been
completely removed.Wilson
When you do that, David, with the Mohs technique, you are doing
that work yourself?Leffell
Yes.  One of the unique things about the Mohs technique is
that the Mohs surgeon is trained to do the surgical procedure to
map the tissue but also to examine it under the microscope, and one
of the advantages of this, and I think it is also one of the
reasons that accounts for the high cure rate of the Mohs technique,
is you have an unbroken chain of events from the time that the
surgeon takes the specimen to the time that they evaluate it under
the microscope and correlate the findings under the microscope with
what is on the map that the surgeon has drawn.  Now, I should
point out that Mohs surgeons are not acting as pathologists. 
We are not diagnosing cancer.  We would never operate on a
cancer that does not already have a diagnosis.  We are
following an existing malignancy pattern and we have available to
us, of course, many experts in dermatopathology.  When we
confront complicated cases, we again engage the teamwork approach
in diagnosing complex situations.Wilson
I would like to add that many patients that I have seen over the
years, especially who have had basal or squamous cell carcinomas in
their head, neck area, some of those patients I have seen have had
relatively large operations and have some cosmetically unattractive
scars. When I have talked to these patients, I have found that they
have not had the Mohs procedure, and so I think another advantage,
see if you agree, is that not only do you have a very confident
sense that you have removed all of the cancer, but also
cosmetically, if we can remove less tissue, and with your
reconstructive skills, make things look good, that sounds very
advantageous for a patient, especially if they have a lesion on
their face, for example.Leffell
I cannot comment on cases that are done elsewhere, I can only
comment on the work that we do and on the principles and the
rationale that underlie the Mohs technique.  It is, as I
mentioned,23:35 into mp3 file 
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and as you imply, a technique for tissue preservation. 
Because we are studying the tissue right away, we only have to
remove the malignancy and can spare as much normal tissue as
possible.  What does that mean?  Well, let's just say
that you have a skin cancer that is 7 mm, the size of a pencil
eraser.  If you are going to excise it with conventional
margins, you have got to take about 4 mm on either side, which is
not a lot, and frankly it is very appropriate in many skin
cancers.  So, let me be clear, the Mohs technique is really
indicated for lesions that are high-risk that are in cosmetically
important areas that have been recurrent already where the edges of
the cancer cannot be identified easily by the dermatologist or the
plastic surgeon.  In fact, a vast majority of skin cancers
below the neck do not use the Mohs technique, but as you suggest,
on the face, you definitely want to take advantage of this
conservative, tissue-sparing technique that permits immediate
reconstruction, all in the office setting.Wilson
What sort of follow-up do these patients require?  You have
done the procedure, it has gone well, the patient is going to go
home after the office procedure.  When do you see them back
again, suture removal, and what sort of follow-up program should
they have?Leffell
We work closely with the referring physician, typically if we do
some form of plastic surgery, they return within a week to have the
sutures removed, and then follow-up with the local referring
physician, the local dermatologist.  Very often, at the
request of the local dermatologist, patients that get a lot of skin
cancers or are high-risk, we will co-manage, we will share the
management and I think in those cases patients certainly benefit
and we're happy to do that, but the most important thing that we do
after the surgery is over is try to educate patients about what
they need to do themselves going forward, and that includes an
aggressive program of sun protection.  Sun protection includes
the regular use of sunscreen with a sun protection factor of 30 or
higher and that includes protection against ultraviolet A waves,
so-called broad-spectrum.  Sometime this year, we expect to
hear from the FDA on a revised monograph that will define new
labeling for sunscreen bottles.  It is true that all of this
is very complicated and the listener, the consumer, often is not
clear, it is generally thought that the higher the SPF number, the
better, and that is not necessarily true, in a practical
sense.  Regular application of sunscreen is important, wearing
a brimmed hat, avoiding the sun during the peak hours of 10 a.m. to
4 p.m., certainly, if you are responsible for young children,
ensure that they are well protected from the sun since so much of
lifetime sun exposure is acquired in childhood.  The most
common thing we hear from people is, well, doc, I am 50, wasn't
most of this damage done when I was a kid?  And the answer is,
yes, but fortunately, people now are living so long that 50 is
yesterday's 30, and I think that we need to make sure that people
realize that they need to take care of their skin and protect it
from the sun because we are all going to be living a very long time
and we can definitely forestall additional damage by ensuring that
we protect ourselves against the harmful effects of ultraviolet
radiation.Wilson
David, is glass protective, if I am in my car with the windows up,
is that enough?Leffell
That is a very complex question and you think there would be an
easy answer.  On the one hand, the film that is used in
automobile glass to make it shatterproof does provide UV protection
and we27:39 into mp3 file 
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generally consider automobile glass to be somewhat UV
protective.  On the other hand, we see patients that come in
with predominantly left-sided skin cancers and they do not live in
England, and clearly they are exposed to sun.  I think that
from my perspective, automobile glass is protective, but it is very
difficult to say explicitly that you do not need to have any type
of sun protection.Wilson
Jumping back to this sentinel lymph node procedure that you had
mentioned, obviously, everything we have discussed thus far can be
done in the office, relatively easy for patients, they go home with
a sentinel lymph node procedure, is that something that is done as
an outpatient as well?Leffell
I am glad you asked that, because increasingly the sentinel lymph
node biopsy procedure has become the mainstay of the management of
many types of melanoma, Merkel cell carcinoma, in some cases,
breast cancer, and other malignancies, and it is a hospital-based
procedure, it is done in the operating room by individuals such as
Dr. Faries that are experts at performing it, and it is one of
those techniques where experience matters.Dr. David Leffell is the David Paige Smith Professor of
Dermatology and Surgery and Deputy Dean for Clinical Affairs 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.