Dermatofibrosarcoma protuberans (DFSP)

bstar said:

Follow up
ALIZA: First I would say follow your instincts with this,we've seen misdiagnosis,we've seen doctors passing it off as nothing, we've seen doctors who did'nt know anything about DFSP and were likely too proud to admit they did'nt know. I have the utmost respect for the doctors and the medical profession, and if it was'nt for them I just may not have survived,however we need to be intelligent and not willing to sit back if things don't seem correct.Its your right to seek a second opinion and have peace of mind.

Myself,I am going for the 6 month checkup in April. I asked the Doctor (my reconstructive surgeon)if I should get a CT Scan or an Ultrasound prior to the visit so we can see what
the area looks like now, he said no it was'nt necessary, the physical inspection and his
verbal consultation will tell him what he needs to know, I trust him,he's the one who knows
exactly what was done,how the area was repaired,every muscle,every fiber,vein,tissue.
He knows exactly what it should look and feel like after 6 months,he's done hundreds of these cases so I trust his intelligence and wisdom. If I had pain I would be insisting
on more comprehensive testing for my own peace of mind.

There were no answers given here but I hope what I've stated will give you some idea how
you should approach your peronal concerns and make a judgement call for your own particular
surgery and follow up exam.Please feel free to comment and keep in touch through this medium.

bstar

New to DFSP
My diagnosis of DFSP was accidental. I had a hard 3cm nodule in the middle of my forehead, and a smaller one closer to my hairline. My dermatologist said it could be a cyst or lipoma, but because of the size and location, she wanted me to see a plastic surgeon. He took one look, said "osteoma", a benign bone tumor, and scheduled an endoscopic removal on 4/21. His plan was to make an incision in the hairline and go in under the skin to remove the "osteoma". However, when he went in, there was no bony growth, but what he thought was a thickening of the periosteum. Thank goodness he took a biopsy.
He gave me the results of the biopsy on my follow up appointment on 4/30. Like many the stories I have read from many people on this site, I was thunderstruck. The plastic surgeon said I was the 3rd case of his career, so his knowledge and experience is not vast. I went back yesterday, 5/14 to receive the results of the immunostaining (CD34). I have been doing my homework, and asked about the Moh's procedure. He feels that he cannot do it because since he went in and "released the periosteum, and disturbed the tissue plane", so he has no idea where the margins would be. He says he could end up taking a huge chunk out of my forehead.
He mentioned radiation therapy, but says that could be difficult on the forehead. He is willing to refer me to a radiation oncologist or any other specialist. I live about 100 miles from Philadelphia, and he says UPenn has people who have seen more than a handful of cases.
His final feeling, though, was that we should just adopt a "wait and see" approach. I said yes to this at the appointment yesterday, but 24 hours later, I am more confused than ever about what to do. Like I have read on several posts, people are reassured that DFSP is slow growing and rarely metastasizes, but why should anyone with this diagnosis gamble on that?
bstar and JC seem to have lots of advice, and I sure could use some!

Rock_n_Remission said:

Keep in touch!
Thanks Rebecca! I am bald now and actually loving it. lol I'd love to say that I might never grow my hair out again as a choice. Except I just found a few days ago that because the tumor extends from my scalp to my skull, they're recommending radiation after surgery. That lessens the chance that I can have any sort of plastic surgery that will avoid a quarter of my head being bald. Either way for now, with my new (and only) tattoo, I feel like a rockstar. lol I had my last day of work on Friday and I am apprehensive about my income but I am doped up on prozac and anti-anxiety meds as well as becoming very adept at filling out financial assistance applications. I've become very lonely since my closest "friends" have accused me of only talking about cancer. Apparently I've become the cancery cancer obsessed b!+c#. :-( First Surgery on Thursday. Let the games begin.
What happened with your followup visit. Has your DFSP returned?

new to this myself
I was injured at work in march, and when I had a hematoma removed from the right side of my skull and found the remnants of a pesky tumor i'd had for awhile. I'd had tumors in my neck before and didn't think much about having another one. when my surgeon sent the tissue to be examined, the results came back as dfsp. again, i'm seeing a recurrin comment about how rare this is. i just had an apt with a dr that i feel just wasn't very helpful. i'm at a point that between the workers comp and the cancer diagnosis that i'm ready to say forget having yet another surgery on my temple. where do u go to find a surgeon that actually knows what this is?

bstar said:

nannylewis
I replied on the 16th the day after your post but for some reason it did'nt get onto the site so I'm sorry about that. Seek out a Mohs surgeon who has extensive knowledge and experience with DFSP. The area affected often determines the surgical procedure he takes.
Here is an example: I traveled 800 miles as the crow flies to see the surgeon who would
perform Mohs Micrographic surgery for me. I full well anticipated having it done then an there and in most cases would have.
As a background,my surgeon does literally hundreds of Mohs surgeries a year and he is so experienced with this he can tell almost exactly what is likely to happen and how to proceed after 20 minutes of examining.As it turned out he said "NO" I won't be doing Mohs for you today it is too involved and we need a team of doctors for the follow up reconstruction. On the surface it was'nt that noticable but he knew it was going to be involved.

Bottom line 3 months later I had Mohs surgery with that surgeon which took 4 sessions over 5 hours and was then admitted to the Hospital and the next morning at 7am
a team of doctors did the reconstruction over a 12 hour period.
The reason I'm sharing this is to show you this well experience Mohs surgeon knew what was coming next after he started his surgery. That is the kind of surgeons are needed with DFSP. Gleevec is being used more and more as pre-op treatment to shrink the tumour before
surgery in some cases to lessen the invasiveness of the the actual Mohs, and also as a post-op treatment to lessen the likelyhood of anything missed. I personally was'nt perscibed Gleevec by the surgeons, I asked about it in passing but they were very confident in there surgical procedures to the point they said 99% or more success.

I hope these comments have helped you for your own peace of mind. Get your proper rest,
its important to healing and being strong.I was'nt sleeping very well after diagnosis because of worry. For the first time in my life I took a sleeping pill each night before bed,it helped a lot. After surgery about a week later I stopped taking a sleeping pill each night and haven't since,that was 9 months ago.

Bstar

Now...some 'peace of mind'
Bstar
Thanks for your reply. The last two weeks for me seemed to last for 2 years! I've been researching DFSP extensively. I heard about the Facebook group, and checked that out, and it was there that I found some names of experienced doctors near me (about 100 miles away - a stone's throw compared to your 800).

Since then I have had appointments with a Moh's surgeon, a facial reconstructive plastic surgeon, and a surgeon who does microvascular skin grafts. All these doctors are associated with John's Hopkins in Baltimore, where they see about 2 cases of DFSP a month - that's a lot for something this rare! At my appointment with the Moh's surgeon, he biopsied the smaller nodule near my hairline to rule out whether it too was DFSP. When those results come back, probably in the next day or two, they will schedule the Moh's.

The bad news is that will not be the end of it. Since they know the periosteum is already involved, they will need to cut out a portion of the bone and do a graft of skin taken from my forearm. I can't describe how I felt when they told me this, but then I don't need to describe it to you, bstar, because you have already walked this walk.

Thank you again for letting me know that I'm not the only one out there that has to deal with this. I've read your posts and you are incredibly strong and upbeat. I'm trying to do the same.

bstar said:

Here are two links,Pip on Facebook has been helpful in trying to locate Surgeons in certain geographic areas, this is new but check it out.

http://dfspsupport.squarespace.com/discusion-page/post/1092475

http://www.facebook.com/?tid=1278219317168&sk=messages#!/group.php?gid=91657360264&v=wall

The first site is a new site for DFSP
The second site is the Facebook group which many belong too and info is shared.

Bstar

9 year old son just diagnosed with dfsp
My son had a nickle sized bump on the top of his foot for only about 2 months when we had it removed (took me that long to get him an appointment). They determined it was dfsp, and admitted him 3 days later to do a wide local resection. They removed the skin and tissue on the entire top of his foot, from the bottom of his ankle to the top of his toes, and all the way deep down to the muscle and tendons. He had a wound vac on for 5 days, and then they did a skin graft off this thigh. Surgeon was confident that he got it all.

We went yesterday for our first quarterly re-check with his Oncologist at Childrens Healthcare of Atlanta's AFLAC Cancer Center. Chest ct scan was clear, and his MRI looked good, Dr said. He had originally told us after the surgery, that all the margins looked clear, but then told us yesterday that he may have 'missed' a report that shows the depth margin was not totally clear. They went all the way down to his tendons, so they really had no other way to go any deeper. As it is, my poor kiddo now has a severly indented foot, where the very thin skin graft did not fill in all the tissue they removed from the top of his foot. Dr is suggesting we meet with the pediatric radiology Oncologist to discuss our options. Has anyone else done radiation with much success? My thought is, why do radiation before we even know for sure that has re-occured? I know all about the high reoccurance rates, but I feel like it's a double edged sword here. If we do radiation now, the dfsp may not come back, BUT he may develop another kind of sarcoma years from now. A more serious sarcoma possibly. If we don't do it, the dfsp may come back, and then we may have to do radiation then, or possibly another surgery. Oncologist seems to think they can't go any deeper though, and the next step would be amputation. We want to try EVERYTHING possible before we get to that point! I feel like we should just watch it, and continue scans and mri's every 4 months, and if it returns, THEN we can look at radiating.

We talked about the Gleevac pill, and Dr didnt seem too confident in it. He said some dfsp's will become resistant to it, and he would need to take the pill the rest of his life. He is only 9 years old, I could handle him taking a pill the rest of his life if it keeps this cancer away! Also, who knows what kind of advancements in medicine they will discover over the next 50, 60 years?

How is everyone else doing that has had surgery? We are 4 months out, and healing fairly well....Brendan was able to wear his first pair of Nikes when school started last month

Melissa

chevypro101 said:

I'm a survivor! I was diagnosed 2 yrs ago with dfsp. Wide excision is what is recommended to treat this type of sarcoma. Other than surgery the only other option out there is radiation therapy. This cancer has an extremely high recurrence rate so that is why they need to be sure to get it all.

I had the wide excision of my dfsp which happened to be located on my left leg. A plastic surgeon is who performed my surgery and after I was healed from the surgery I follow up with an oncologist and my PCP. The down fall to the surgery (in my opinion) was the skin graft. It was painful to me. Where they removed the dfsp from was fine; it was where they took the skin graft from is what caused me the most pain.

The providers I went to were at Johns Hopkins Bayview Medical Center in Baltimore, MD. I highly recommend my plastic surgeon, Michele Shermak. Dr. Shermak discussed my case with my oncologist and other providers at Johns Hopkins. That is how they determined the best treatment plan for me. I was thoroughly explained all my options and what was recommended.

Hi,
My 8 year old niece was
Hi,

My 8 year old niece was diagnosed last week and has it on her right leg, they took x rays on friday and we are afraid it is in her lungs although they haven\t confirmed cause its the weekend, they did leave a message stating her surgery would be Tuesday.

Is this a cancer that can be fought off long term or should be even more worried ???

my 14 year old nephew has cancer for 10 years but not this kind so i am wondering if you know cause i am not finding much i understand. i live in Canada.

Any info, good or bad but honest would be helpful.


\thank \you

Digger33 said:

DFSP
Hi everyone
I'm in a similar boat. I had a lump removed from my upper middle back in July that was supposed to be a simple, benign Lipoma. It turned out to be DFSP. The lump measured 4CM in the report. The lab report said it was low-grade which I learned means the rate of cell division was relatively low. I have no idea how long it was there. I'm 38 and if my wife didn't see it, I may never have noticed it. It was flat, round, a slightly squishy and it had a little purplish color. It was somewhere between the size of a quarter and half dollar. It was painless.

I ultimately went to Fox Chase Cancer Center in Philadelphia PA to see Dr. Watson. He has treated 30 patients for DFSP. He told me that 28 times it stayed localized and was treated successfully. Two times it spread with one fatality.

He sent me for a CT scan and chest X-ray to make sure it hadn't spread. Luckily, they were normal. On Sept 2nd, he performed a wide excision on my back. He took as much tissue as he thought he could take and still stitch me closed without requiring reconstructive surgery. He cut an ellipse in my back. My location is fortunate as it allows for the removal of a large amount of tissue. He said the back is not a common occurrence for this. He said his goal is a 2CM margin on all sides. He will accept 1CM but nothing less. I got my lab result on 9/10. The left and right margins were clear but the top and bottom was <1CM. The bottom was only 1MM or less. He recommended follow-up surgery which I'm scheduled for 9/23. This time, it will require a plastic surgeon too who I will meet on the 17th. I trust Dr. Watson. He really seems to know his stuff and he is really familiar with DFSP so if you are in the Northeast US, he is someone to check out. The plastic surgeon is part of the Fox Chase team so they work it together and have done others together.

For me, the toughest part of this is the mental anguish of having a type of cancer with a high recurrence rate. He took a big hunk out of my back the first time and my family and I thought we were done and were quite surprised to hear the lab results weren’t clear. I know there are a lot more difficult types of cancers and I'm thankful this one is usually highly treatable but it still is a mental grind. I'm glad to hear that some of you have been cancer free for a long time and hopefully forever. Dr. Watson said we will do periodic CT scans in the future. I think he said every 6 months for the first year but I haven't been able to think about much past the surgery. The thing I find interesting is that he will place little markers in my back (pins) to outline the area removed so that if it does come back, the CT scan will show the pins and he will be able to tell where it came from relative to his cut.

There is another web site worth checking out where you can read about other people with DFSP. http://www.rarercancers.org.uk/

I wish you all luck and health with your treatment.

-Steve-</p>

DFSP on the back
My son also has his lump on his back. I posted on the DFSP part of this board. He already had the tumor removed a couple of weeks ago and the pathology report came back as DFSP. He goes back for more extensive surgery next week. His tumor was the size of a golf ball so I am terrified they are going to take a huge amount from his back?? The tumor had been growing for 10 years. They had diagnosed it as a cyst and he even had it drained 5 years ago. I pray it hasn't spread. This site is so informative and helpful. I just wish people came back an updated their postings. My son may have a spot on his hip also that he hasn't even shown to the dr. yet. I am worried after reading postings that it may be too late to perform the MOHS since his tumor has already been removed and that it was so large. If anyone has any knowledge of this it would be helpful.

Thanks,

A worried Mom

VanessaK said:

My Dermatofibrosarcoma protuberans
In June of 2005 I took it upon myself to see a surgeon to have a growth in my upper left arm removed. For more than a year I felt a growth in my arm- I went to my GP and was told that it was a fibroma- a fatty tissue growth and not to worry. It didn't bother me but I felt that it was growing-a year later I started having tingling sensations running down my arm and stabbing sensations so I decided to get a second opinion. I was 27 years old. I had the initial visit with the surgeon and was told that it was one of 3 things- 1 of which he did not suspect-but had only treated 3 times in his 25 year career- a sarcoma. I didn't think twice and had no idea that I would get the call that it was in fact cancer. I went in to have the mass removed on 6/25/05- a few days later I was told that pathology came back confirming it was a dermatofibrosarcoma protuberans and that I needed another surgery for a wide excision because the margins were not clear. I found this e-mail that I sent to my boss that week:
I had to have a CT scan done on Monday, when I arrived for that they told me that my doctor also requested a bone scan; with that test I had to have a radioactive material injected in me and then come back for the scan.I had to return on Tuesday with an empty stomach, I arrived on Tuesday at 11 am and had the radioactive isotope injected and I had to return 3 hours later to have the scans done. Following the procedure i was radioactive for the next 48 hours and was advised by the technician to try and seclude myself from people, especially small children and pregnant women. On Thursday I had the surgery, the doctor told me that they would be taking an inch on each side plus 1 inch deep. They gave me general anesthsia and I woke up an hour later in a lot of pain and a very large bandage. It turns out that they cut me about 8 inches long and they removed the fatty tissue all the way down to the muscle. My bandage was getting pretty saturated so I changed it on Saturday. When I saw what they did to me (even though the most important thing is that it's all gone) I was shocked and upset. I have 29 staples from the middle of my upper arm to the crease on my underarm. It is really painful on both ends because it got so swollen and the staples are being pulled by the swelling. The painkillers help but they make me really sleepy.
I went annually for the past 5 years to have a chest CT to ensure that it had not spread and 6 years later I am sarcoma free- fingers crossed that I will not have to deal with this issue again. Love and positive vibes to anyone going through this now.

6 months after initial surgery..Recurrence!
I was diagnosed with Bednar's Tumor, a subgroup of DFSP, indicated by a blue tint. It's usually subcutaneous, and mine was close to the facsia. My surgeon did a wide excision on my left deltoid. The tumor looked to be pea size and like a broken bluish blood vessel. After the wide excision, my dermatologist oncologist didn't get a good enough margin, .2mm. They had me do radiation at a high dose for 30 days. I finished in the end of February. My surgery was done in November, but due to a bad infection, they had me wait until the end of December to begin radiation. I was not to thrilled with my Dr, so I went to MD Anderson for my follow-up visit. That was in the end of March. He wanted me to wait a month before getting a MRI to check to see if it was gone. This week I noticed a blue mark exactly the same as the original tumor, in the same location. Apparently, the radiation did not work as planned. I live in Utah, and have lost much confidence in the Dr's here, thus the trip to MD Anderson. I am very small to begin with, and since they took out over 15" of skin, I'm not sure how surgery will work, assuming they will excise it again, this time, I'm assuming with the MOHS procedure. Could anyone advise me on what to ask my Dr's concerning CT or PET scans. I'm so worried that I've had a recurrence after 6 months, that I want to check for any other parts it ma have affected. I'm young (32) and in very good health(I own a Pilates studio). I want to be much more proactive this time, since the first round I just did what I was told even though the Dr's seemed to be inexperienced with this rarity. Also for those who have experienced recurrence, how many? I'm hesitant to take Gleevec. I'm thinking that it may be a reality though. Since my tumor is not large in size, where some DFSP patients have such large tumors that the meds help shrink it, so that doesnt apply to me. Any thoughts, similar experiences or advice would help! Also, I read in a Japanese study, that the recurring cancer is more aggressive, and because of that, it puts your statistics higher for metastasis. Does anyone know of the statistics for recurring patients? I'm so glad I found this forum!

Dermatofibrosarcoma proturberans
I was just diagnosed and you are right there isn't much info available. However, what I have found is that moh's microscopic surgery is THE BEST treatment. I have also read that wide excision has a higher rate of recurrence than MOHs. MOHs surgery has a 99 per cent cure rate. Google this surgery and do your research. It is easy to be railroaded into other treatments but stick to your guns. I wish you all the best. I too am having the same issue with the doctor that diagnosed me. I have seen her for all of 10 minutes and she won't even make time to speak with me. I only get to speak to her nurse or secretarial staff. It is seriously frustrating because I can only get a referral for a MOHs surgeon through her. I am considering going to another dermatologist in another city that will actually speak to me and answer my questions. I too was told to have the wide excision surgery and then have it sent to a pathologist and if there was still cancer then I would get the MOHs surgery. But why go through more than one surgery if you don't have to. as the surgeon removes the tumor and tissue they check it under a microscope and look for cancer cells. If there are still cancer cells then they continue to cut until they have removed it all.

bstar said:

Here are two links,Pip on Facebook has been helpful in trying to locate Surgeons in certain geographic areas, this is new but check it out.

http://dfspsupport.squarespace.com/discusion-page/post/1092475

http://www.facebook.com/?tid=1278219317168&sk=messages#!/group.php?gid=91657360264&v=wall

The first site is a new site for DFSP
The second site is the Facebook group which many belong too and info is shared.

Bstar

Advice
Hi Bstar

I was just diagnosed. What worries me is that this bump under my skin has been there for at least 20 years. My doctors have always dismissed it as some sort of trauma that occurred that I just didn't remember. I had it biopsied in June because it got a little bigger and sore to the touch. In would like to know what I should do the dermatologist that I was referred to will ot call me. My diagnosis was basically done by phone by one of her staff that could not even pronounce it. I want to get referred to the MOHs clinic in Toronto. She won't call me back and is basically insisting that I see a general surgeon and have it removed and have MOHs of it comes back or the pathology indicates they didn't get it all. I have been reding a lot about scar tissue and multiple surgeries. How do I get her to refer me to the clinic. Also are there any other tests I can get my family doctor to send me for in preparation? My concern is that because this lump has been around for a long time, there is more to it than what can be seen on the surface. Is this a legitimate concern. Also I am worried about the level of care I am receiving in my city. It took almost two months to hear from her office after the biopsy. My family doctor called me about three weeks before the dermatologist and told me that I must follow up with the dermatologist. She had the results for a number of weeks before calling. I am very frustrated and worried and could really use some advice.

I am very happy that I found this site.

Moemcn said:

Dermatofibrosarcoma proturberans
I was just diagnosed and you are right there isn't much info available. However, what I have found is that moh's microscopic surgery is THE BEST treatment. I have also read that wide excision has a higher rate of recurrence than MOHs. MOHs surgery has a 99 per cent cure rate. Google this surgery and do your research. It is easy to be railroaded into other treatments but stick to your guns. I wish you all the best. I too am having the same issue with the doctor that diagnosed me. I have seen her for all of 10 minutes and she won't even make time to speak with me. I only get to speak to her nurse or secretarial staff. It is seriously frustrating because I can only get a referral for a MOHs surgeon through her. I am considering going to another dermatologist in another city that will actually speak to me and answer my questions. I too was told to have the wide excision surgery and then have it sent to a pathologist and if there was still cancer then I would get the MOHs surgery. But why go through more than one surgery if you don't have to. as the surgeon removes the tumor and tissue they check it under a microscope and look for cancer cells. If there are still cancer cells then they continue to cut until they have removed it all.

DFSP on forehead
I was just diagnosed with DFSP. Tumor removal was 9/14/11. It was located on my forehead above my left eyebrow near the hairline. Doctor called me 9/20 with results. Cleveland Clinic contacted me 9/21 to schedule the first consultation for 10/3/11. I have been reading as much information as I can. I was told that I would need reconstructive surgery. I believe MOHs surgery is the best solution, however, based on the location and additional unknowns, I have to be prepared for other alternatives. Please keep all information coming!