Dermatofibrosarcoma protuberans (DFSP)

DeaVan · January 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.

AuthorUnknown · January 2007

Hello,

You may want to contact the American Cancer Society's National Cancer Information Center. Cancer Information Specialists are available 24 hours a day and can assist you with questions regarding side effects from treatment. They can be reached at 1-800-227-2345.

Take care and be well,

Dana
CSN Dana

chevypro101 · March 2007

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.

Csmith · June 2007

Hello,

Which lab performed the original diagnosis? Was the immunohistological stain CD 34 performed?

Did you have an MRI with gadolinium before surgery.

Did you recieve a second diagnosis from another lab?

Chris

dbutler · August 2007

I was diagnosed with DFSP on July 9 and had the surgery on July 16, 2007. My surgeon made made a wide incision around the tumor until he got clean margins. From what he says the tumor has a five percent chance of coming back. The survival rate is 90 percent. The only thing I can not find an answer for is, will this made me more susceptable to other types of cancer? If anyone knows more about DFSP please post. This a rare disease and there is not a lot of info on the net or my the american cancer society.

steetanya · June 2008

I have been diagnosed with DFSP, I would like to discuss this cancer with someone else who has it.Since it is rare there are not a-lot of cases out there.If anyone has had this please contact me at t_steeeno@verizon.net I am very worried

lis232 · July 2008

steetanya said:
I have been diagnosed with DFSP, I would like to discuss this cancer with someone else who has it.Since it is rare there are not a-lot of cases out there.If anyone has had this please contact me at t_steeeno@verizon.net I am very worried

I'm a recent survivor of DFSP. I was diagnosed just about a year ago to the day. I had a "nodule" on my abdomen and my PCP sent me to a plastic surgeon to have it removed. I had the option of having it biopsied first but given it was bleeding at that point I just wanted it removed. The tumor was not terribly big but I had about a 5 inch scar. the pathology report showed it was DFSP but I didn't have clear margins. I had another surgery - a wide excision which took even more tissue. I now have a 13 inch scar across my abdomen. A plastic surgeon performed this surgery with a pathologist in the OR. I also consulted with oncologists prior to surgery. My surgery was successful, I did need one more reconstructive surgery about 3 months after the wide excision. The recovery was extremely difficult as I could not sit up on my own after two major surgeries. I was hospitalized for a bit less than 3 days.

My long term care has been with a sarcoma clinic with regular physical exams and tests and I'm in remission almost a year now.

My best advice for anyone with DFSP with its high recurrence rate is to work with a plastic surgeon or a dermatologic surgeon to remove the tumor and do reconstructive work. But it's just as important if not more so to seek out a cancer/oncology facility that has a sarcoma clinic for your ongoing care during remission. Be proactive, don't wait.

coolme · July 2008

I was diagnosed with this in June this year. I've had the surgery and have been told of a medicine (Gleevec) that I have to use. Has anyone used this yet? Are ther side effects? I'm worried! I'm told this is a rear thing. I'm looking anywhere for as much information as I can get.

amyr329 · August 2008

coolme said:
I was diagnosed with this in June this year. I've had the surgery and have been told of a medicine (Gleevec) that I have to use. Has anyone used this yet? Are ther side effects? I'm worried! I'm told this is a rear thing. I'm looking anywhere for as much information as I can get.

i was diagnosed last august
i was diagnosed last august and had the mohs procedure to excise it. i just found out yesterday its back and i just read about gleevec. i read about it on emedicine.com, and also have the same qeustions as you. i have a 7cm scar on my neck from the first excision and probably will have another scar the same size and do not want to have to keep going through this every year. if you get any good info on the drug therapy please let me know

Rancho · September 2008

amyr329 said:
i was diagnosed last august
i was diagnosed last august and had the mohs procedure to excise it. i just found out yesterday its back and i just read about gleevec. i read about it on emedicine.com, and also have the same qeustions as you. i have a 7cm scar on my neck from the first excision and probably will have another scar the same size and do not want to have to keep going through this every year. if you get any good info on the drug therapy please let me know

Mohs procedure and recurrence
I opted for the more radical surgery, CCPDMA. It stands for "complete circumferential and peripheral deep-margin assessment." I had clear pathologic margins after this procedure that was done on September 2, 2008. I will be having a body scan later in the month. At this time I will not need radiation or Imatinib mesylate therapy. Access www.nccn.org for DFSP. It discusses clinical practical guidelines for our cancer.

Good luck,
Rancho

Digger33 · September 2008

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-

Rancho · September 2008

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>

30 Patients
Hi Steve,

I am curious about the two patients where the DFSP had spread. Do you know where the primary lesion was located? Into which organs did it migrate? How was the one treated that had spreading and is still alive? Sorry for all the questions. The medical center where I was treated, has seen very few cases of DFSP. Luckily, my surgeon is a personal friend with whom I work and I knew going into the procedure what a great dissectionist he is. My incision was quite large also (about 10 inches) and was in my groin. My wife also noticed how different the tumor it looked; it was like your description. I minimized the whole thing but she insisted I had someone look at it as I work at a med center. Since surgery, my walking has been a little labored and I will be off work until Oct. 6. I am a runner and plan to work hard to be able to run at least one half marathon before the new year.

I will fire a few prayers your way on the 23rd. I plan to check out the rare cancers website.

Craig

Digger33 · September 2008

Rancho said:
30 Patients
Hi Steve,

I am curious about the two patients where the DFSP had spread. Do you know where the primary lesion was located? Into which organs did it migrate? How was the one treated that had spreading and is still alive? Sorry for all the questions. The medical center where I was treated, has seen very few cases of DFSP. Luckily, my surgeon is a personal friend with whom I work and I knew going into the procedure what a great dissectionist he is. My incision was quite large also (about 10 inches) and was in my groin. My wife also noticed how different the tumor it looked; it was like your description. I minimized the whole thing but she insisted I had someone look at it as I work at a med center. Since surgery, my walking has been a little labored and I will be off work until Oct. 6. I am a runner and plan to work hard to be able to run at least one half marathon before the new year.

I will fire a few prayers your way on the 23rd. I plan to check out the rare cancers website.

Craig

Info
Craig,
I hope you are back to full speed real soon. It sounds like we both need to thank our wives for pushing us as I too tried to dismiss it as nothing. I didn't think it was any big deal. I almost didn't have it removed as the doctor said it was mostly a cosmetic issue and I didn't want to be tied up in the summer.

As far as the two people who had it spread, it was tough for me to hear the story. The one fatality was a guy my age. It started in his shoulder and it was removed. When it returned, he said it traveled pretty far which is unusual for DFSP. I think he said it ended up in his lungs but I'm not 100% sure. He definitely said one of the favorite place for soft tissue sarcomas to travel is to the lungs which is why he sent me for the CT scan but I don't know if that was the case with the guy who died. I don't recall what he said about the other person. I'll ask him again on Tuesday when I see him.

Thanks for the prayers. Tuesday is a little more intimidating for me after my consultation with the plastic surgeon. Given the location on my back and the fact that this is my third surgery so there is not much excess skin remaining, he is going to make multiple crescent shaped incisions down my back to rotate skin around after Dr. Watson removes the additional tissue. He will leave in multiple drains too which doesn't sound fun. It sounds like I'll be laid up for a few weeks too. As soon as I feel up to it, I'll put up a post after my surgery to let you know what I find out.

If there is any other info I can provide, let me know. Feel free to ask me anything as I’m happy to help. Given the rarity of this thing, it’s helpful for me to chat with other people in similar situations. I'm rooting for you to hit your marathon goal. Hopefully this will be a distant memory for both of us by next year.
-Steve-

Rancho · September 2008

Digger33 said:
Info
Craig,
I hope you are back to full speed real soon. It sounds like we both need to thank our wives for pushing us as I too tried to dismiss it as nothing. I didn't think it was any big deal. I almost didn't have it removed as the doctor said it was mostly a cosmetic issue and I didn't want to be tied up in the summer.

As far as the two people who had it spread, it was tough for me to hear the story. The one fatality was a guy my age. It started in his shoulder and it was removed. When it returned, he said it traveled pretty far which is unusual for DFSP. I think he said it ended up in his lungs but I'm not 100% sure. He definitely said one of the favorite place for soft tissue sarcomas to travel is to the lungs which is why he sent me for the CT scan but I don't know if that was the case with the guy who died. I don't recall what he said about the other person. I'll ask him again on Tuesday when I see him.

Thanks for the prayers. Tuesday is a little more intimidating for me after my consultation with the plastic surgeon. Given the location on my back and the fact that this is my third surgery so there is not much excess skin remaining, he is going to make multiple crescent shaped incisions down my back to rotate skin around after Dr. Watson removes the additional tissue. He will leave in multiple drains too which doesn't sound fun. It sounds like I'll be laid up for a few weeks too. As soon as I feel up to it, I'll put up a post after my surgery to let you know what I find out.

If there is any other info I can provide, let me know. Feel free to ask me anything as I’m happy to help. Given the rarity of this thing, it’s helpful for me to chat with other people in similar situations. I'm rooting for you to hit your marathon goal. Hopefully this will be a distant memory for both of us by next year.
-Steve-

Steve,

My incision will show definite scaring but because he was able to fold and pull the skin, plastic surgery was not needed. My surgeon had to do some creative overlapping but opted not to place a drain. The way he sutured it, it drained by itself anyway for the first week. At time the sutures were removed 15 days after surgery, I had a serotoma (fluid in the wound) which needed further draining (about 10cc).

I was also told that the lungs were a target for spreading. I'm looking ahead to my body scan and I feel pretty confident. I have been walking 1/2 mile a day with just a little pain. I ordered some new running shoes, Cubs cap and wind shirt but had to promise my wife I would not do any running till at least mid October. ( Although I have been on the west coast a long time, I am from Chicago and have always liked the Cubs).

I will pass on any new info I acquire. I am a clinical scientist, so if I can help you with information let me know. Try to enjoy the rest. I know plenty of work will be there when I get back but that's O.K. Although my colleagues have missed me, they have been very supportive and want me to take my time. I plan on it.

It is nice to have a person to talk to and for sure this will be behind us as we continue to be cancer survivors.

Craig

Digger33 · September 2008

Surgery update
Craig,
My surgery went fine. Slight curve ball in that they kept me in the hospital overnight to run some antibiotic through my IV (along with some morphine). I have two drains that will be in until next Wed. The first time they drained about 50ccs each but by the time I left today, one was down to 10 and the other down to 5. I should get my lab results then or sooner but Dr. Watson is very confident he got it all this time as he took a very large chunk from my back. He told me not to worry. They want me to take an oral antibiotic this week too to minimize the risk of infection.

I did ask him about the two patients that had it spread. In both cases, it went to their lymph nodes first. In the person who died, it went to the lungs after his lymph nodes. It did not spread to the lungs in the other person who is still alive. That’s as far as we got with the conversation as there were a bunch of people around working on the surgery prep.

I’ll shoot you a post when I get my test results. I hope your scan goes well.
-Steve-

Rancho · September 2008

Digger33 said:
Surgery update
Craig,
My surgery went fine. Slight curve ball in that they kept me in the hospital overnight to run some antibiotic through my IV (along with some morphine). I have two drains that will be in until next Wed. The first time they drained about 50ccs each but by the time I left today, one was down to 10 and the other down to 5. I should get my lab results then or sooner but Dr. Watson is very confident he got it all this time as he took a very large chunk from my back. He told me not to worry. They want me to take an oral antibiotic this week too to minimize the risk of infection.

I did ask him about the two patients that had it spread. In both cases, it went to their lymph nodes first. In the person who died, it went to the lungs after his lymph nodes. It did not spread to the lungs in the other person who is still alive. That’s as far as we got with the conversation as there were a bunch of people around working on the surgery prep.

I’ll shoot you a post when I get my test results. I hope your scan goes well.
-Steve-

Steve,

I'm glad to hear your surgery went well. Antibiotics sound like a good idea. The last thing you want is an infection. Thank you for the info about the two patients. Lymph node involvement is the scary thing about our cancer. I seems like for both of us, it will not be a problem.

The radiology department took about 300 raw images of my torso. Collating the images and having them read by a radiologist will take about four days. I see my surgeon again on October 1 and will get the results of the scan at that time. I'll let you know the results. I may be going back to work on October 6. We'll see.

One challenge I've noticed is stamina. My body is used to more exercise and it isn't getting enough. I'm working on it and know it will improve. My wife has been wise and so supportive. Usually I'm the problem solver and "go to" person.

Heal well and avoid any backslapping friends!

Craig

Leelee Jordan · September 2008

Digger33 said:
Surgery update
Craig,
My surgery went fine. Slight curve ball in that they kept me in the hospital overnight to run some antibiotic through my IV (along with some morphine). I have two drains that will be in until next Wed. The first time they drained about 50ccs each but by the time I left today, one was down to 10 and the other down to 5. I should get my lab results then or sooner but Dr. Watson is very confident he got it all this time as he took a very large chunk from my back. He told me not to worry. They want me to take an oral antibiotic this week too to minimize the risk of infection.

I did ask him about the two patients that had it spread. In both cases, it went to their lymph nodes first. In the person who died, it went to the lungs after his lymph nodes. It did not spread to the lungs in the other person who is still alive. That’s as far as we got with the conversation as there were a bunch of people around working on the surgery prep.

I’ll shoot you a post when I get my test results. I hope your scan goes well.
-Steve-

Thanks for website info
Steve,

Thanks for letting me know about this forum. I looked several years ago when I was diagnosed and I'm pretty sure there wasn't one for DFSP at that time.

Just to let everyone know a little about me I'm a 48 year old female with a mixed up diagnosis for DFSP. When I finally found out on my own by reading my pathology report that was three and a half years old. I had been told that it was benign by a surgeon that couldn't read. I had MOH's surgery to my right hip one day (3 times to surgery) and then followed up with more surgery and reconstruction the very next day. Total seven inches across and down to the muscle. I am fortunate to have good dr's now but wasn't so lucky back in 2002.

I'm doing well now (2 1/2 years) after surgery.

ttyl

Digger33 · October 2008

Test Update
Craig / Sandra,
I got good news in that my lab results were clean. No additional DFSP was found. The plastic surgeon removed my drains today too which is a big relief. Last thing is stitches out next week and I should be good. Thanks for the support.

I also found another website that had a bunch of DFSP posters. It is a little tricky to navigate since it has other form of sarcomas too but if you play with their search engine, you can find a bunch of DFSP folks. You need to get to their discussion board first. Link attached.

http://www.sarcomaalliance.org

Good luck everyone.
-Steve-

DaisysMom · October 2008

Digger33 said:
Test Update
Craig / Sandra,
I got good news in that my lab results were clean. No additional DFSP was found. The plastic surgeon removed my drains today too which is a big relief. Last thing is stitches out next week and I should be good. Thanks for the support.

I also found another website that had a bunch of DFSP posters. It is a little tricky to navigate since it has other form of sarcomas too but if you play with their search engine, you can find a bunch of DFSP folks. You need to get to their discussion board first. Link attached.

http://www.sarcomaalliance.org

Good luck everyone.
-Steve-

a question
Hi Steve, Are you going to have to have radiation now that the surgery is over with?
Congrats on getting the stitches out next week!
Best wishes,
DaisysMom

Rancho · October 2008

Digger33 said:
Test Update
Craig / Sandra,
I got good news in that my lab results were clean. No additional DFSP was found. The plastic surgeon removed my drains today too which is a big relief. Last thing is stitches out next week and I should be good. Thanks for the support.

I also found another website that had a bunch of DFSP posters. It is a little tricky to navigate since it has other form of sarcomas too but if you play with their search engine, you can find a bunch of DFSP folks. You need to get to their discussion board first. Link attached.

http://www.sarcomaalliance.org

Good luck everyone.
-Steve-

Test Update
Steve et al.,

Steve, I am pleased about your good news. Keep healing! My scan came back with a spot on my liver. Lungs, spleen, pericardial and pleural areas, viewable lymph nodes, pancreas, gall bladder and adrenals were all clear. I have an MRI for my liver this Saturday (Oct. 4) and will have results later next week. I feel healthy and plan to start running mid October. Hopefully, the spot is nothing but I am glad to be having more confirmatory testing. I will let you know about my results. I am going back to work on Oct. 6.

Take care,
Craig

Digger33 · October 2008

DaisysMom - At my first surgery with Dr. Watson, he said the latest research shows that radiation for DFSP is only marginally effective so he does not support radiation when he can get clean margins. The plastic surgeon who closed me up this time was surprised I wasn't getting it. I have a follow-up with Dr, Watson next Thursday and I was going to ask him about it again. Right now, there are no plans for it.

Craig - when I had my CT scan, it showed 2 spots on my liver and 3 spots on my right lung. All were < than 5MM. Doctor Watson considered them nothing special and probably due to normal wear and tear of living and breathing in an industrial society. He said they don’t even biopsy anything until it is at least 1CM but he will look for changes in any future CT scans. The folks who did the CT scan recommended all kinds of follow-up tests but Dr. Watson said they usually do that just for precaution. Hopefully, yours will be nothing too. Good luck.

-Steve-

Dermatofibrosarcoma protuberans (DFSP)

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