kellie harvey

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!!

thanks

Kel

j

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
<http://groups.google.com/groups?as_epq=Dermatofibrosarcoma%20Protuberans&sa fe=images&ie=ISO-8859-1&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

sharon

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

Please Sharon, That would be great!

Kellie

j

there's an article here about it http://tinyurl.com/xzmr 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
congenital cases have been reported. There is equal *** distribution in
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

j

I've got some (maybe) good news for you Sharon,
There's a rare cancer mail list at ACOR
http://listserv.acor.org/archives/rare-cancer.html (240 subscribers)

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

http://sarcomaalliance.org/main.html?ArticleId=141
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
http://www.rsroc.org.tw/English/Ejournal/article/V26/N4/260406.pdf

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
http://www.geocities.com/sampyroy2000/DFSP.html
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

j

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 http://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 http://www.emedicine.com/derm/topic97.htm
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