Dermatofibrosarcoma Protuberans

Kellie Harvey said:

I had DFSP last october on my left clavicle, I have found a spot not too far away from the
original site of the cancer, My Doc has sent an emergency referral to get it looked at. has anyone
been through this, can they tell me what goes on, I know no one who has had dfsp and want to talk
to someone who has had experience of it. bad or good news I want to know everything!!

Hello Kel, Unless they've spelled it wrong (or called it something else like fibrosarcoma or
sarcoma), there's been 3 in the Google archives <groups.google.comgroupsOpen ↗
1&as_scoring=d&lr=&hl=en>

Anita's (posts are) on this newsgroup (and had DFSP removed) but haven't heard in a few days. She's
also battling thyroid cancer. J

"Kellie Harvey" <[email hidden]> wrote in message

I have a support volunteer for the Rare Cancer Alliance who has had personal experience with this
disease. If you want to have her contact you, please let me know.

Take Care, Sharon

Kellie Harvey said:

Hi

I had DFSP last october on my left clavicle, I have found a spot not too far away from the
original site of the cancer, My Doc has sent an emergency referral to get it looked at. has anyone
been through this, can they tell me what goes on, I know no one who has had dfsp and want to talk
to someone who has had experience of it. bad or good news I want to know everything!!

there's an article here about it tinyurl.comxzmrOpen ↗ Wayne State U Dermatofibrosarcoma
protuberans (DFSP) is a low-to-intermediate-grade malignant tumor that accounts for approximately 6%
of soft tissue tumors in children and in adults. DFSP is diagnosed most frequently during the second
to fifth decades. Fewer than 200 cases of DFSP in children and only 20

adults and children, but a female predominance in congenital DFSP. This tumor has a relatively low
metastatic potential. However, local recurrence after excision is common.

there's more there J

Please Sharon, That would be great!

Kellie

Sharon said:

"Kellie Harvey" <[email hidden]> wrote in message

I have a support volunteer for the Rare Cancer Alliance who has had personal experience with this
disease. If you want to have her contact you, please let me know.

Take Care, Sharon

Sharon said:

"Kellie Harvey" <[email hidden]> wrote in message

I have a support volunteer for the Rare Cancer Alliance who has had personal experience with this
disease. If you want to have her contact you, please let me know.

Take Care, Sharon

I've got some (maybe) good news for you Sharon, There's a rare cancer mail list at ACOR listserv.acor.orgrareOpen ↗
cancer.html (240 subscribers)

The (potential) bad news, on further searching this cancer archderm.ama-archderm.ama-Open ↗
assn.org/cgi/content/abstract/114/4/599 Dermatofibrosarcoma protuberans with lymph node and
pulmonary metastases

sarcomaalliance.orgmain.htmlOpen ↗ The lungs are the most frequent site of
metastases, but metastases to brain, bone, and heart have also been reported.[4] Metastases usually
in cases occur within 6 years.

Something similar can affect the breast
rsroc.org.tw260406.pdfOpen ↗

HTH, FYI and FWIW J

Hello Kelly A little of my history: Papillary thyroid Cancer diagnosed Jun 2003, Modified neck
dissection and total thyroidectomy Nov 2003,

DFSP discovered during surgery (was suspected to be a sebaceous cyst!) but turned out to be DFSP.

Anyway I have been reading a lot and some idea what this is. I am sure you know all the basic stuff
from links that J sent you.

DFSP: can recur locally. does not metastasize easily (but I dont believe in statistics anymore, I am
probably the ONLY person other than my grandmon in the planet with both DFSP and thyroid cancer !!!)

First line of defense: surgery. Two schools of thought for this
1) Mohs Micrography: where the take slices of tumor and during surgery itself look at it in the
microscope. This way they hope to make sure that they get negative margins and remove all the
offending tissue without removing good tissue. Les trauma. this is current gold standard
2) Wide excision: they take out a few cms of tissue around the tumor margin and hope they have got
it all. I am unfortunately in the situation of having to go through wide excision because my
primary DFSP is removed and my head-and neck surgeon prefers to do wide excision. He is probably
not trained in Mohs micrography that bein the field of Dermatologists. but I cannot afford to go
to a dermatologist because I have had modified neck dissection an total thyroidectomy and dont
want oo many cooks in that vulverable part of my neck. So I have to trust my head- and neck
surgeon to do his best.

DFSP-FS: this is amore aggressive form of DFSP, it has something called a fibrosarcomatous change.

DF: this is considred benign.

They have to make sure it is not the FS variant of DFSP. DFSP tissue can undergo special
immunohistochemical staining to decide what kind it is. DFSP regular stains for CD34- 50-100% of the
cells, but DFSP-FS stains for CD-34 less than 50% of the cells. The other stains that DFSP stains
are positive for Vimentin and p53 and negative for Factor Xiiia DFSP tissue has low or scattered
mitotic figures, DFSP-FS has high number of mitotic figures
geocities.comDFSP.htmlOpen ↗ DFSP occurs when the (PDGF) Platelet Derived Growth
Factor gene fuses with COL1A1 gene. The PDGF gene can no longer do its job correctly because its
fused with something else. PDGF is a potent cell division stimulant of fibroblasts (connective
tissue cells), smooth muscle and other connective tissue. If you have distant mets, they will
probaby do chemotherapy, but I think they will just do surgery because it may have locally recurred
for you. I found something called STI571 (imatinib) that is used to treat Chronic MyeloProliferative
Leukemia, regularly. this thing is supposed to inhibit PDGF mediated growth. Anita (not a doctor-
just reading enough to pass a couple of med schools exams 😉 )

I forgot to mention that it surgery does not get rid of it completely and it keeps recurring,
apparently they will try radiation as next step before chemotherapy.

Anita

Quoted message said:

<snip>
2) Wide excision: they take out a few cms of tissue around the tumor margin and hope they have got
it all. I am unfortunately in the situation of having to go through wide excision because my
primary DFSP is removed and my head-and neck surgeon prefers to do wide excision. He is
probably not trained in Mohs micrography that bein the field of Dermatologists. but I cannot
afford to go to a dermatologist because I have had modified neck dissection an total
thyroidectomy and dont want oo many cooks in that vulverable part of my neck. So I have to
trust my head- and neck surgeon to do his best.

DFSP-FS: this is a more aggressive form of DFSP, it has something called a fibrosarcomatous
change. <snip> If you have distant mets, they will probaby do chemotherapy, but I think they will
just do surgery because it may have locally recurred for you.

Hi Anita, Good to hear from you. I'm not sure where Kelly is because Sharon had tagged her for her
rare cancer website and I had also referred both of you to the www.acor.org (rare cancer), so
hopefully Kelly will be around soon.

So yours did not get a clear margin? or a recurrence? It was my understanding that: DFSP is slow
growing low-grade, superficial sarcoma AND does not respond to chemo
emedicine.comtopic97.htmOpen ↗ Medical Care: Currently, chemotherapy is not used in the
treatment of DFSP. Radiation therapy has had a limited role in the past, but, recently, it has been
used as an adjunct to surgery. Radiation therapy may be recommended for patients if the margins of
resection are positive or for situations where adequate wide excision alone may result in major
cosmetic or functional deficits. Close follow-up care after radiation therapy is warranted because
some of the tumors may become more []

So you may want to question if it ever gets to the point of someone offering chemo, because I don't
think thyroid cancer responds to chemo either, so seems to me it might be a waste of time and energy
(unless you can find me something reliable that says otherwise) or maybe it's the aggressive kind
that they use chemo on? I don't really know because I'm not an expert.

When's the next surgery? I'm wondering if radiation therapy is better soon after or if it's better
to wait it out for another recurrence, because I guess there's limits to how much radiation therapy
can be given.

How's your thyroid levels ? Stabilizing? Feeling somewhat better now? Still in a different city? Did
you get home for the holidays? Hope so :-) (sorry for all the questions).

How incredibly unlucky you've been by having two cancers and in the same area.

So just stopped in to give you a hug. ( ( ( Anita ) ) ) J - not an expert

Hello J, Big hug back at ya. thanks. nice to know you are reading my mails.. I am back home and
doing quite well. the neck mobility issue is still there, but not so much, it has
stabilized...Thyroid levels ok and TSH is getting there- was 1.2 last checking.

As for DFSP: Nope no clear margins. 🙁 They say lateral margins are positive, i think they will have
to remove a LOT, because I remember feeling that my skin was extra fleshy (just a mit nothing anyone
other than me would notice) on the left side of the neck where the DFSP was.

Now I am waiting for RAI BUt something is going on, pain in the base of the skull left side which
radiates up into the head sometimes and left eye pain. Nothing that will make me run to the ER
but... I know enough to know that if I FEEL that something is wrong it is.

About the Chemo I read studies from Uppsala university and even in the US that imatinib or STI-571 I
think its caled gleevec or is that the company was successfully used in DFSP. They dont routinely do
chemo on patient I realize, I hope I did not mislead Kelly.

I want to avoid radiation therapy as much as possible.

Nobody really knows what terrible things radiation does to the body, ofcourse it kills the cells,
BUt it can also destabilize DNA in other cells, which may have chugged along fine inspite of having
a mutation or two, ie., push them over the edge.

I prefer surgery, I think the body knows how to handle trauma, we have evolved to protect ourselves
in that event, radiation in huge doses is so unnatural...

Today being MLK day I havent been able to call ANY doc for appointments, have a ton of them to
see... I am feeling GREAT though- almost no depression this thyroid thing is AMAZING. So my body
needed to be at this level all along...

Anita

Quoted message said:

As for DFSP: Nope no clear margins. 🙁 They say lateral margins are positive, i think they will
have to remove a LOT, because I remember feeling that my skin was extra fleshy (just a mit nothing
anyone other than me would notice) on the left side of the neck where the DFSP was.

Now I am waiting for RAI BUt something is going on, pain in the base of the skull left side which
radiates up into the head sometimes and left eye pain. Nothing that will make me run to the ER
but... I know enough to know that if I FEEL that something is wrong it is.

I prefer surgery, I think the body knows how to handle trauma, we have evolved to protect
ourselves in that event, radiation in huge doses is so unnatural...

Glad to hear you are back home and the thyroid levels look very good..

You could poke around here maybe about Gleevec
nci.nih.govgleevecOpen ↗
centerwatch.comdru735.htmlOpen ↗

#13
asco.org1,1003, 12 002514 00 18 0016 00 19 00375 00 21 0011,00.aspOpen ↗

I used sarcomas and 17. Low Recurrence Rate for Wide Excision of Dermatofibrosarcoma Protuberans So
let's hope the next surgery and maybe radiation therapy fixes this once and for all ,so you can get
on with your life and give the doctors and hospitals a break. :-)

For some reason, when I see that chemo, I think the cancer has to have a good blood supply. Plus I
do not understand your thinking about radiation therapy. It's "judicious radiation therapy". Sure it
maybe does something to the cels near where it's pointed, but chemo is systemic (ie affects all
areas and organs of the body), so I'd rather go with radiation therapy, but hey it's your cancer,
not my body, so the decision will be up to you. And if we're out to lunch on our information, we're
misinforming everyone who reads this. It's up to them to check anything that's posted with their own
doctors/oncologists, right?

I'm sorry you're having pains and some feelings that something's still not right. The neck area can
be chaotic, I've got tendon and ligament problems are on the left jaw joint side and it extends pain
down the left into the neck, into the face/cheek, jaw and the ear and up into the skull. (so it's
hard to tell what causes what especially when a person's had so many surgeries as you). And a
pinging feeling up the side of my left neck,no idea if it's a nerve or ligament or blood vessel,
it's just an annoyance to me.

It's a worry isn't it? And sometimes doctors don't feel (with their hands) the differences we can
feel ourselves.

Sounds like you'll be very busy, but do keep in touch when you can. ( ( ( Anita ) ) ) J

Quoted message said:

As for DFSP: Nope no clear margins. 🙁 They say lateral margins are positive, i think they will
have to remove a LOT, because I remember feeling that my skin was extra fleshy (just a mit nothing
anyone other than me would notice) on the left side of the neck where the DFSP was.

Now I am waiting for RAI BUt something is going on, pain in the base of the skull left side which
radiates up into the head sometimes and left eye pain. Nothing that will make me run to the ER
but... I know enough to know that if I FEEL that something is wrong it is.

About the Chemo I read studies from Uppsala university and even in the US that imatinib or STI-571
I think its caled gleevec or is that the company was successfully used in DFSP. They dont
routinely do chemo on patient I realize, I hope I did not mislead Kelly.

I prefer surgery,

Cancel my last message. I just read Gemini's reply to Kelly about Gleevec. Imatinib Mesylate Hugs J

J, My mistake was assuming Imatinib was a kind of chemo therapy drug. I WILL NEVER ASSUME ANYTHING
AGAIN. However plausible it sounds to me. Yep, I have a whole bunch of links for imatinib (STI-
571) sounds promising, but they also had people who developed resistance... I guess there will
always be those.

I am hoping somewhere in this line of research for cures of other cancers they find something that
works for anaplastic thyroid carcinoma (the kind my grandmom had) as well... God knows there is
almost no research going on for anaplastic thyroid cancer...so only hope that something happens
serendipitously...

Anita

Quoted message said:

Cancel my last message. I just read Gemini's reply to Kelly about Gleevec. Imatinib
Mesylate Hugs J

anita said:

My mistake was assuming Imatinib was a kind of chemo therapy drug. I WILL NEVER ASSUME ANYTHING
AGAIN. However plausible it sounds to me. Yep, I have a whole bunch of links for imatinib (STI-
571) sounds promising, but they also had people who developed resistance... I guess there will
always be those.

I am hoping somewhere in this line of research for cures of other cancers they find something that
works for anaplastic thyroid carcinoma (the kind my grandmom had) as well... God knows there is
almost no research going on for anaplastic thyroid cancer...so only hope that something happens
serendipitously...

Hello Anita, After I saw your post, I looked up anaplastic thyroid cancer at Medline
nlm.nih.gov000352.htmOpen ↗
1) very rare
2) very aggressive and invasive
3) Most people do not survive longer than 6 months. So I'm sure it's not that no one cares, it's
just that there's so little time. For instance, for clinical trials.

I'm just glad that's not the type you have, because golly, you wouldn't even be here, due to all the
delays in getting back home for surgeries and treatments.

As to DFSP, we'll have to hope they can get clear margins this time. I'll watch for your updates and
treatment plans, while you continuing researching.

Keep in touch Anita, I think of you often. Hugs J

Just an FYI - here is a link to a hospital currently conducting a clnical trial on Anasplatic
Carcinoma of the Thyroid . . .

irelandcancercenter.orgClinicalTrials H&K.htmOpen ↗

Kim C

"J" <[email hidden]> wrote in message "]news:[email hidden]...

Quoted message said:

anita said:

My mistake was assuming Imatinib was a kind of chemo therapy drug. I WILL NEVER ASSUME ANYTHING
AGAIN. However plausible it sounds to me. Yep, I have a whole bunch of links for imatinib (STI-
571) sounds promising, but they also had people who developed resistance... I guess there will
always be those.

I am hoping somewhere in this line of research for cures of other cancers they find something
that works for anaplastic thyroid carcinoma (the kind my grandmom had) as well... God knows
there is almost no research going on for anaplastic thyroid cancer...so only hope that something
happens serendipitously...

Hello Anita, After I saw your post, I looked up anaplastic thyroid cancer at Medline
nlm.nih.gov000352.htmOpen ↗
1) very rare
2) very aggressive and invasive
3) Most people do not survive longer than 6 months. So I'm sure it's not that no one cares, it's
just that there's so little

time.

Quoted message said:

For instance, for clinical trials.

I'm just glad that's not the type you have, because golly, you wouldn't

even be

Quoted message said:

here, due to all the delays in getting back home for surgeries and

treatments.

Quoted message said:

As to DFSP, we'll have to hope they can get clear margins this time. I'll watch for your updates
and treatment plans, while you continuing researching.

Keep in touch Anita, I think of you often. Hugs J