Dermatofibrosarcoma protuberans (DFSP)

Ontario48 said:

Good Morning Anne...

You are most welcome.  I had a hard time with the lack of information I was getting from my doctors so I did a lot of research and reading on my own.  Mostly medical articles.  A recurrence does put you in another bracket in my opinion.  Did you have any other screening...MRI/chest scan.  This type of cancer typically goes to the lungs if its going to spread.  I would insist on a chest scan since you've had a recurrence and you have muscle involvement.  This is considered deep seated and needs to be monitored. 

No doctor can guarantee that there will not be a recurrence.  They can only predict based their surgical plan and resulting pathology reports. This is why you need to be screened closely for the next five years and then annually after that.

We are very lucky in Canada as we have access to specialist through our public health care system.  Mount Sinai and Princess Margaret hospitals in Toronto are the best in Canada.  PMH is dedicated to cancer treatment and MS is the "mecca" for sarcoma.  Sarcoma is very tricky and very rare. 1% of all cancers and DFSP is 1% of that group. DFSP has a very low metastic rate and lower death rate so we are lucky for that.

Its important you find a doctor that you feel 100% confident in.  Once you have that, the stress level will go down.  You can also ask for your pathology reports.  I found that helpful for me, the more information I had in hand the stronger I felt.

Keep me posted Anne...Joanne

Dear Joanne. Thank you so much once again. I will not hesitate forwarding your expertise and experience to the doctors, I will be consulting in the future, if that is ok? I must try to be clearer when I formulate myself (not a native speaker of English so some misformulations are bound to happen, but, hopefully, I make good sense). As far as I understood, the cancer had not spread to the muscle. They "simply" removed the upper part of the muscle in order to make absolutely sure they got it all; wide excision. But since it had 2 cm clear margins, that must mean that the "tentacles" of the sarcoma did not yet have the chance to spread to the muscle. I have an appointment at the Doctor on 24 April and I will insist on a thorough examination and biopsies of every single area I find suspicious looking. 

I have NEVER been screened. Yes, I had a breast screening years back and everything was clear but that's it. No MRIs or chest scans this time; Only wide excision and microscopic examination of the removed flesh/skin with a 2 cm clear margin. Thank you once again, you are so kind for sharing and being so informative. It is very helpful to share and learn through people in the same boat:) All best and take care, Anne.

Ontario48 said:

You make good sense, lol, ; )

Hello Anne,

My heritage is Ukraine.  My auntie speaks broken english so I can translate the "misformulations" just fine...LOL.  The screening part is the problem.  Since this cancer is so rare and has a low metastic rate most doctors will not do all the screening.  Unless of course you use a private practice and they sign you up for everything to make the $$.  Public wants to save $$.  You must insist and its your right to do so.

I would absolutely insist on a chest CT Scan to ensure nothing is there.  Do know if it has gone there its still very cureable.  I don't want you to be afraid of it.  From what I have read most doctors do nothing except remove the first go round.  If you have a recurrence or the more agressive type DFSP-FS (Fibroscarmotas), then they tend to do full screening. That being said, I have read a few medical articles where the site was small, no FS component and no recurrence but spread to the lungs.  Its about your piece of mind.

When I say musle involement it doesn't have to be diseased muscle.  Most grow parallel to the skin surface...horizontal.  Ours grows into the core, verical.  I had a six inch incision but it when down to the muscle.  Three layers of removal, 3 layers of stitches.

Have I asked...is your doctor a sarcoma specialist?

Follow your instinct.  You know your body better than anyone.

Cheers for now, Joanne

2-3 cm is the recommended margin so thats good they did that.

 

 

etc.  When this occurs they tend toward WLE. 

In any account its

For now, thank you soso much, Joanne. 

No, my doctor is not a sarcoma specialist at all, so will insist on one who is from now on. I have found the name of a sarcoma specialist in my city (Aarhus, Denmark) - that is VERY fortunate. Will see if I can get a reference to him. 

I have a LOT of work to do in order to get the right treatment it seems. I cannot thank you enough for your help and your advice and I will not hesitate to tak action ASAP. All best and take care, Anne.

Ontario48 said:

Good Luck...

and keep me posted.  If you email  me from here I will get a notification in my in box. I am not on here much.

You are on the right road!  All will be fine...cheers for now, Joanne

Thank you!! Yes, will stay positive. Will do:) Cheers, Anne

I was recently diagnosed with DSFP after having a lump removed from my shoulder/collarbone area. Several years ago I had a lump on my shoulder that grew to around the size of a golf ball when I had it removed. It turned out to be a non-cancerous fibrous tumor. After the surgery, I developed some bad keloid scars. A few months ago I noticed another lump, this time the size of a marble. In the time between when I first noticed it and when it was removed, about 6 weeks later, it had grown to the size of a large marble. This time, however, the biopsy came back as DSFP. There was no indication on the surface other than the lump that it was cancer. Despite using a wide excision, not all of it was removed. After the cancer diagnosis, I've been sent to see an oncologist who is planning another surgery. He's not using Mohs surgery due to the size and the fact that there is a fair amount of scar tissue. I'll be getting surgery again in around a month, this time it will likely include skin and possibly muscle grafts. Fortunately, it didn't penetrate to the bone. I'm not sure how worried I should be. The oncologist said often when they get to the size mine grew to the center can turn into another form of cancer that does metastasize though fortunately, that didn't happen in my case.

aaronw1970 said:

Just came across this thread

I was recently diagnosed with DSFP after having a lump removed from my shoulder/collarbone area. Several years ago I had a lump on my shoulder that grew to around the size of a golf ball when I had it removed. It turned out to be a non-cancerous fibrous tumor. After the surgery, I developed some bad keloid scars. A few months ago I noticed another lump, this time the size of a marble. In the time between when I first noticed it and when it was removed, about 6 weeks later, it had grown to the size of a large marble. This time, however, the biopsy came back as DSFP. There was no indication on the surface other than the lump that it was cancer. Despite using a wide excision, not all of it was removed. After the cancer diagnosis, I've been sent to see an oncologist who is planning another surgery. He's not using Mohs surgery due to the size and the fact that there is a fair amount of scar tissue. I'll be getting surgery again in around a month, this time it will likely include skin and possibly muscle grafts. Fortunately, it didn't penetrate to the bone. I'm not sure how worried I should be. The oncologist said often when they get to the size mine grew to the center can turn into another form of cancer that does metastasize though fortunately, that didn't happen in my case.

Sorry to hear that you have had to join this elite club, but welcome.

I wont write too much as I tried before and my replies are not getting through but ill try again.

Mohs is what I eventaly had and 5 ys on im doing very well. I have never had MRI in relation tio my DFSP. But I had one of Australias best sarcom surgons so I was completly confident of his abilities and he also preformed all the pathology testing. 

So hopefully by now the new members have secured a good team of knowledgable medical  people and you are all on your way to full recovery.

Please dont hesitate to ask question or get back on here tovent your feeling & concerns. If anyone understands  what your going through its us here. I for ine found my chats here during those early days were comforting and very informative.

Take care 

From

Nat

natmcg said:

Newcomers to dfsp.

Sorry to hear that you have had to join this elite club, but welcome.

I wont write too much as I tried before and my replies are not getting through but ill try again.

Mohs is what I eventaly had and 5 ys on im doing very well. I have never had MRI in relation tio my DFSP. But I had one of Australias best sarcom surgons so I was completly confident of his abilities and he also preformed all the pathology testing. 

So hopefully by now the new members have secured a good team of knowledgable medical  people and you are all on your way to full recovery.

 

Please dont hesitate to ask question or get back on here tovent your feeling & concerns. If anyone understands  what your going through its us here. I for ine found my chats here during those early days were comforting and very informative.

 

Take care 

From

Nat

I'm glad yours is in remission.

In my case, they had already removed the tumor before determining it was DFSP. It was the size of a large marble and was growing rapidly. Before the surgery, the thought was that it was the same non-cancerous tumor that I had removed a couple years ago had returned so there was no biopsy performed beforehand. Only afterward, the pathology report came back as DFSP and also that they didn't get it all.

I finally spoke with an oncologist at Stanford and it sounds like in my case Mohs surgery won't work. Next week I have an appointment with another surgeon, a plastic surgeon since both will be involved. It sounds like there will be skin and muscle grafts. Fortunately, it does not appear to have metastasized or gone into my bone though they're talking about doing some X-rays to make sure it hasn't spread to my lungs. Fortunately, the oncologist has dealt with DFSP before. On the other hand, the tumor did seem to grow rather quickly in my case and suddenly which has me worried because it sounds like when this happens it tends to be a lot more dangerous.

I should have been paying more attention and it's possible I might have caught it before it became the size of a small marble under my skin. There was no discoloration above it. After my first surgery, there was a lot of scar tissue in the area and some bad keloid scars that were being treated with injections of steroids. From my research, it sounds like this likely developed due to the previous surgery on my shoulder.

RichR64 said:

Hello

I had what was believed to be a cyst removed on oct 5th 2017 after biopsy I was diagnosed DFSP.as of today half of the incision is still not healed.i was wondering if anyone else has had this problem.my surgeon recommends a wider excision.i have a appointment to discuss the MOHS procedure and was wondering if anyone could help me decide which procedure I should have.also last July I had a partial nephrectomy.i was told cancer was caught in time and did not reach arteries.is this a coincidence to have 2 cancerous growths removed in a little over a year?

Sorry to hear that you have had another cancer diagnosis . As far as I am aware there is no reason to relate DFSP with other forms of cancer. (But I could be wrong). I have however read of others having DFSP and different cancers at some point in their lives.

You haven't said how large your current wound is but we all heal differently so if your concerned why not get a GP or nurse to have a look just to rule out infection etc. 

As to which type of surgery to have next both are very successful at eliminating remaining tissue affected by this type of cancer. Due to the location, size and my surgeons experience I had Mohs. But its up to you to discuss all options with your Drs and to be comfortable with what they are proposing. My only advice would be ask lots of questions, get a surgeon that has experience with DFSP or at very least a sarcoma specialist.

My excision was closed without the need of a skin graft due to the location ( just under my breast)but due to the depth & there not being anything inbetween my skin & muscle it was very sensitive for quite some time.

And last but not least....once again DFSP has been misdiagnosed at first sight.

It absolutely infuriates me that Drs still just assume that all lumps on top or under the skin are cysts or scars. A needle biopsy could save so many people time and worry.

Anyway no use dwelling on that, the good thing about this type on cancer is that in the majority of cases once it's all surgically removed there is no further treatment needed 

Take care and good luck .

I'll check in soon to see if you have posted again

Bye for now

Nat.

natmcg said:

Hi aaronw1970

 

It sounds like you have an experienced team working with you & your on the right path to eliminating your self of DFSP once and for all. 

Hopefully you will heal this time without the past issues.

 

When I was going through my experience with DFSP I at times felt very confused as to how worried I should be about this one in a million cancer diagnosis. Five plus years on and it's still being misdiagnosed and there is no ryme or reason as to why we get it.

Iv given up searching the internet for information , the more I read the more variables  I  found on  possible outcomes.

Now I'm just great full that it's behind me

As you will be too.

Wishing you a speedy recovery

I'll check in to see if you post again.

 Nat.

 

I underwent a wide excision on 11/21 where they cut out a section of my shoulder around 8 inches long and 1.5 inches wide. The pathology came back that they got it all, though I need to get an MRI every three months for follow-up appointments. My shoulder and neck are extremely tight but the pain isn''t too bad, I think in part because my shoulder is numb. I should start physical therapy in a couple of weeks. Fortunately there were no skin or muscle grafts but it was close to that.

The worst part before the operation was not knowing what I'd be in for afterwards. I would have gone back to work this week if I hadn't come done with a cold. I find I'm limited with what I can do with my left arm but hope the PT will help.

Hopefully I'm over the worst of it now and won't have to deal with any more surgeries. I think what I took to be scar tissue from a previous surgery to remove a non-cancerous tumor was actually the cancer since they removed all of it.

Fortunately I still seem to have a good range of motion of my left arm as well so hopefully there won't be any long-term impact from this.

The worst part of this whole ordeal was psychological in not knowing what I'd wake up to.

-Aaron

natmcg said:

Hi aaronw1970

 

It sounds like you have an experienced team working with you & your on the right path to eliminating your self of DFSP once and for all. 

Hopefully you will heal this time without the past issues.

 

When I was going through my experience with DFSP I at times felt very confused as to how worried I should be about this one in a million cancer diagnosis. Five plus years on and it's still being misdiagnosed and there is no ryme or reason as to why we get it.

Iv given up searching the internet for information , the more I read the more variables  I  found on  possible outcomes.

Now I'm just great full that it's behind me

As you will be too.

Wishing you a speedy recovery

I'll check in to see if you post again.

 Nat.

 

I have also done some research. It sounds like it can form due to a burn or other injury when a couple of chromosomes get cross-linked at just the wrong place where the gene to create collagen gets linked to a growth promoter gene according to Wikipedia. In my case, I believe it was a result from a previous surgery I had to remove a non-cancerous tumor from the same location. I had a bunch of what looked like keloid scars where I had the surgery that were being treated with steroid injections to shrink them when I noticed the lump under my skin. I think the keloids were actually the DFSP. I'm recovering well, though my neck and shoulder are quite tight from all the removed tissue and I have started physical therapy.

natmcg said:

How time flies.

Hi everyone, 

I hope everyone had a safe & happy festive season.

Also I hope recovery and healing is going well.

Glad to see we haven't had any new comers .

 

Warm Regards

Natalie

Unfortunately I am the latest new comer.  I had a "cyst" on my lower back for over 4 years now.  Every doctor I saw blew if off saying it's nothing to worry about just a normal cyst.  Well I finally decided to have it taken off and was diagnosed on February 8, 2018.  I was in shock, horror and disbelief.  I think I zoned out after he said the words "rare Cancer".  I've been doing a lot of research on it, but I'm finding conflicting stories.  On my pathology report it said it "appears" to be completely out, however no mention of margins.  I am terrrifed that this has spread.  I feel like my doctors aren't taking this seriously because they keep telling me not to worry, everything will be fine.  I am awaiting an appointment with Memorial Sloane Kettering, but unfortunately they don't think this is an emergency so I have to wait until April 11th.  I'm just wondering, if this is such a slow growing cancer, how long does it take to reach other organs in your body?  They say it's rare that it spreads, but I've read numerous stories of people who say that it has affected other organs in their bodies.