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Dermatofibrosarcoma protuberans (DFSP)

DeaVan
Posts: 2
Joined: Jan 2007

Has anyone had or no someone that has/had this type of sarcoma? I know it is very rare, but I am having a very hard time understanding what to do. I was diagnosed in November and immediately scheduled for surgery to remove this from my arm. The surgeon only spent about 10 minutes with me prior - and just kept telling me how rare it was and it had to be removed. Since then, I was told they did not get it all and I would have to have more surgery and also additional biopsies(sp) for two other possible areas. I refused to have the surgery until speaking with at least an oncologist. The oncologist sent me back to my primary care physician to have the biopsis, wants to do the surgery with a plastic surgeon for required skin grafts, but also has stated he isn't positive the pathologist was correct in the original diagnosis and wants someone else to evaluate it because it is so rare. My primary care physician scheduled me for the biopsis, but changed her mind that day and now won't do the biopsis since I have an oncologist. I've now (since I can spell it) done the homework and am more confused than ever on why I'm being told surgery is the only option and no one seems to be in charge of this or willing to really work with me and explain what is going on. Sorry for the long winded message, but I am really scared, confused and looking to be proactive in making sure I am fully informed and going in the right direction.

mykidisthebest
Posts: 1
Joined: Mar 2013

I was 12  when I found a spot on my lower back that was bothering me.  My mother looked at it and said "come on,  it's not like it's cancer or anything!"   It kept bothering me so  she took me to 5 different doctors over the years.  Every time I went in they told me it was a cyst and it will go away.  It was annoying having this pain.  Finally, a doctor  told me that it doors look like it's a cyst but it shouldn't be hurting so he decided to take it out. I  had local anesthesia  and they cut in and said it wasn't a cyst but looked like scar tissue.  I was 17 when they found that it was cancer.  They told me they had to research it because they never heard of it before. I had 3 surgeries before they  told me it was gone.  

 

 

 

I am now 33. About 8 months ago I felt this spot on my upper back  by my shoulder blade.   It's growing.  So I am headed back into the doctor to get a biopsy  because the spot looks the same and  feels exactly like the one that was in my low back.  I had the connective  tissue  type 'Dermatofibrosarcoma Protuberans. I was told it  would only come back in the same spot. Has anyone heard of it coming back in other areas? 

 

 

natmcg
Posts: 111
Joined: Jun 2012

Hi, Im writing this in the hope that it will make you feel a little better in some way. First to answer your question ...no I haven't read of DFSP reoccurring in a new area(not that I can remember anyway) and yes reoccurring DFSP seems to always come back around the scar area. That's why we are told to keep a close check on our scars. Your story is unfortunatly so very common,not many Drs think the lumps are anything to be concerned about. It is only when we tell them of the pain, itching or discomfort do they do something and like in your case they cut before doing a biopsy. In that respect I was lucky my the Dr wanted a biopsy before removing it as he is trained in detection of skin disorders as well as being a GP but he really didn't expect it to be cancer let along such a rare one. Your doing the right thing by having a biopsy so keep that in mind. There will be no misdiagnose this time you know exactly what you want it checked for. Remember "its nothing until the Dr says it's something." I'm not making light of your situation. Not at all... 4mths after my final op. I was on holidays and noticed a mole under my left ear. My DFSP was under my left breast and the scar way still quite fresh and I had been told that if it were to come back it would be near or on the scar tissue. And it was very unlikely as I have good clear margins. But...The minute we got home I made an appointment wth my specialist. On the drive to his office I was thinking this thing has to be removed or biopsied asap. But I had worked myself up into a frenzy over a simple mole and its still under my ear(and I still check it every day lol) I'm hoping you will have the same outcome. Take care and try not to worry too much . Nat.

Ontario48
Posts: 54
Joined: Jan 2013

Hi there,

I must say you are an anomaly as far as the statistics go.  A, because you had DFSP so young and B, you had a recurrence elsewhere.  My skin cancer surgeon told me there was a 1% chance of regrowth elsewhere.  So consider this...DFSP is one in a million.  Soft tissue sarcoma in adults represent 1% of all adult cancers and DFSP within that group is "very rare".  And now you represent a 1% regrowth elsewhere.  Not sure where you live but you should get yourself on a study list in your area.  It's important to document such occurrences for this.  We must thank our stars that this cancer is slow growing and typicaly does not produce distant spread. If you get a positive for DFSP again I encourage you to get a chest screen to ensure there is no spread there.  You are at higher risk for this after recurrence.  I would also question how it got to where it went...blood vessels, lymph nodes etc. 

Connective tissue indicates a deep growth which contributes to a higher regrowth rate.  Again, it's important that these types of tumors are documented so the pros can understand them and better manage them.  Since ours, thank god, has a low rate of death it seems it's not taken as serious as other cancers.  I strongly recommend hooking yourself up with a STS (Soft Tissue Sarcoma) specialist.  Dermotologist get on this as it's in the skin but it's not like any other skin cancer.  It's tricky.  The pathology is tricky too.  You want someone who is versed in sarcoma.

I myself have just found a STS specialist and am very interested in the discussion with her.  I will share once that appointment has happened (in May).  We can't fault our docs for not knowing about this.  It just so rare.  Sarcoma study in itself is just getting momentum.

Good luck, keep us posted on your progress if you can.  I am sure we all will find you fascinating.  We all have that "recurrence" cloud over us at the moment.  We are here to help each other.

Cheers...Joanne

Mrdeli
Posts: 2
Joined: May 2013

Hi everyoone, 

Had a WLE surgery. 

Margins came back clear --- which I knew was key, but the margin was too small .2 of a cm. 

My oncologist got me an X-Ray, bloodwork, and a thoracic CT scan with contrast.All clean. 

My oncologist wrote a letter to the plastic surgeon and this week he cut out another (second) WLE that was much bigger than the first WLE. Pathology will be back in a week. I am expecting since margins are clear that there should be zero neoplasm found in the sample this time, right ? 

 

M

 

 

natmcg
Posts: 111
Joined: Jun 2012

Hi, M.

Welcome to the dfsp "one in a million club".To bad you didnt have a choice.
You havnt given a great deal off info, but from what you have written I think you can be confident that you are in the clear.
I had Mohs surgery and although the pathology looked good my surgon decided to remove a bit more to be 99.9% sure that I would not have to have more surgery. So far 1yr on everything is going fine.

Was this a recurrence of dfsp for you.? If not then your medical team seem to be very thourough with your scans and tests.
Have these Drs your seeing delt with dfsp before? Just curious....

Try not to worry between now and getting your results.

Sending Best Wishes for (extra) clear margins.:-):-)

Natalie.

Mrdeli
Posts: 2
Joined: May 2013

Hey Natalie

Yes, I was worried when oncology set up the thoracic CT. I was told it was routine as I had already  been diagnosed. This is the first time I have had DFSP. My plastic surgeon says he has seen DFSP about 20 times in 28 years. He mentioned that people from all over travel to the plastic surgeons they are the front line. My surgeon was not a big fan of mohs for DFSP  unless its in a spot that cannot have tons of tissue removed, like face or neck. Since mine was in the lower forearm he basically said its better to take out the tissue with WLE. I am a bit concerned about percentages WLE vs MOHS but up until now we have been in shock and have been following what they tell us.

I don't know how much experience my oncologist had with DFSP but she picked up on getting a bigger margin the minute I got in her office. She mentioned that since I have cancer the benefits of CT always outweigh the risk going forward forward. I took this observation rather stoically. I have heard via the radiologist that she has no bedside manner but is very thorough. Works for me!

natmcg
Posts: 111
Joined: Jun 2012

Hi M,

I don't think you have anything to worry about in regards to not having mohs. From what I have read and what my surgon told me the main thing is to get it all. And experienced DFSP Drs. know not to skimp  on the clear Margins. Thats why they sent you back to get a better/wider clearence. 

In my case I asked  my GP to  do the first excision even  though  he recomended I  see a specialist.  He removed the tumor and went with recomemded margins but a week  later I was told that the margins were not clear and that now it was obvious that it had spread to the fatty tissue  as well. So having mohs  was more  about avoiding more than one more surgery to get those " Clear Margins" . And They did :-)

And fortunately it hadnt made its way into the muscle tissue. 

So from what you have written it looks like you are in very good hands  and should be hearing good news soon.

Keep Us Posted

Nat.

 

marshal g
Posts: 1
Joined: Oct 2013

I was diagnosed with DFSP 3 months ago.  It began as a lump on the back of my scalp. I mistook it for a cyst of some type and didn't bother to see the doctor for at least 6 months.  When I originally visited a dermatologist he thought/hoped it was a lipoma but as soon as he "punched" the tissue for removal he knew it wasn't.  A biopsy confirmed DFSP and I scheduled an appointment with a Mohs trained dermatological surgeon here in SLC 2 weeks later.  

It was quite the experience.  My wife and I were originally told it would take 1/2 a day of surgery but it had spread much further than anyone anticipated.  The surgeon removed tissue 5X on one day and a 6th time on the following day - he sent us home saying that he would not be able to take anymore tissue and scheduled an appointment for me to see a surgical oncologist.  Fortunately the last Mohs procedure came back with clear margins and I was immediately scheduled to see a plastic surgeon.  4 days later I ended up with 70 staples and 60ish stitches following the flap procedure to close the 12cm x 8cm scalp wound.  2 months later my recovery from the surgery is almost complete.  

I don't know what advice I can offer.  Mohs was the best option for me since the cancer was on my scalp.  No one has recommended chemo or radiation at this point but they want me to see the dermatologist every 3 months for 2 years.  There's lots of scarring, mostly from the reconstruction, but my wife still loves me and I don't have dfsp - so I'll take that for now and hope for the best going forward.

If anyone is in the SLC, UT area - I recommend the UofUtah Medical Center's Mohs Clinic and Reconstruction Team.  The treatment I've received gives me confidence about my future.

Marshal

Cuda340
Posts: 2
Joined: Feb 2014

Hi Marshal, I have a similar tumor on the top of my head. My skull has caused it to grow outward and its pretty large now. Is there any advice you can give me at all. My Mohs surgery is in 2 months. I guess the surgeon is pretty busy. Again any advice would be much appreciated.

periwinkle1979
Posts: 1
Joined: Jan 2014

Hi, I'm 34 years old and was just diagnosed with DFSP a few days ago.  I first noticed a bump under my breast about 6 years ago.  Like many of you, each Dr. told me it was a just a cyst and would probably go away on it's own.  4 1/2 yrs later it had gotten much bigger and super sore since my bra rubbed directly against it.  My Dr. did a surgery in office to remove what he thought was one cyst.  He ended up removing, I believe, 5 total tumors.  He told me they were just cysts that multiply and not to worry.  Well 6 months later they showed up again, only this time I could see three on the surface of the skin.  I went back to my original Dr. who sent me to a surgeon since there was too much scar tissue for him to opporate out of his office.  The surgeon encouraged me to get a biopsy.  It came back positive for DFSP.  I officially meet with the oncologist in a few days, but from what the financial director said, he wants me on Gleevec immediately.  It's such a whirl wind.  To be told for years that it's nothing but a harmless cyst by several doctors only to find out it's cancer....ugh!  After much research, I'm convinced MOHS is the best choice for surgery, keeping my fingers crossed that it'll be a possibility for me.  I'm super thankful it's not a super scary cancer.....but it's still cancer and that has me deeply concerned.  Any of you tried Gleevec?   The side effects sound AWFUL!  Some websites I read say that chemo is helpful and others can it's completely ineffective.  Thoughts?

natmcg
Posts: 111
Joined: Jun 2012

Im sorry you have had such a run around before getting the right diagnoses. I dont understand why the first Dr didnt get the first lumps he removed tested. but that cant be changed so you just have to move on. My story is very similar to yours,even the location is the same. I had discoloration on a small indent under my left breast which I noticed when I was in my 30's. The spot would become sore and itchy, and yes like you my bra made it worse. When a reddish lump started to form I showed  my GP . Even had an ultrasound done, and was told it was just a fatty tissue cyst, nothing to worry about. Two years ago I decided id had enough of the itchiness  and while having a full body skin cancer check I asked the Dr if he could remove these anoying lumps ( I had two by this time). He said a biopsy should be done first. And you guessed it!!  Inocent fatty cysts are cancer......DFSP.   After pursuading the same Dr that I was happy for him to remove the offending lumps asap (as I was due to go on holidays). I ended up having to go to a specialist because to patholagy showed there was more to be removed. Clear margins were not achived ( I should have listened to him and waited for the sarcoma specialist to do mohs.). So finaly mohs was done and two yrs on I have a 6inch scar under my left boob but no DFSP. 

So here are my suggestions to you(no advise im not a qualified person ) Find a sarcoma specialist that has experience with DFSP.. Travel if needs be to see such a person if you can. Its worth the effort, time and money if you do. Hopefully ,mohs will still be an option.

Gleevec is  not something i know about,  except for what iv read in posts here. And I have read that it does seem to inhibit the growth of tumors in cases where surgery is  no longer an option. 

You havent , mentioned where you live.  Im guessing  you are in the USA or Canada.

Im in Australia so its probably no use me recomending any medical people here.

Stay positive, this is going to take some time to work through but its doable.

Sending you best wishes

Natalie..

Cuda340
Posts: 2
Joined: Feb 2014

I was diagnosed with a large tumor on the top of my head. Scheduled for Mohs surgery in 2 months because the surgeon is booked. Has anyone had it on the head and if so can you give me any advice.

natmcg
Posts: 111
Joined: Jun 2012

Hi sorry to hear about your dfsp. My tumors were not on my head so I cant help you with that.  What I can say is dont worry about having to wait for surgery. This is a slow growing form of cancer. The most important issuse (from my point of view) is to have a Dr.who knows what he/she is dealing with, and can offer you answer 5o all your questions. 

There are a few  old posts on this board from people who have had dfsp  on and around  their face and head. From what I remember reading the removal of dfsp is  the same anywhere on the body.they keep going untill they get it all. There could be a a need for skin grafts/ transplants depending on the amount of tissue removal. I was lucky even after having a peice af flesh the size of a small mobile ph removed there was still enough strech in my stomach to pull it all together and stitch it up. 

If you havent already doneit, I suggest you tap on the heading Discussion Boards and have a read of the other posts about dfsp.  I will check in again in a day or two to see if you have been back.

 Bestwishes 

Natalie.

 

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