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Dermatofibrosarcoma Protuberans (DFSP)

Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Nov 19, 2011 11:59 am

I was diagnosed with Dermatofibrosarcoma -  rare form of cancer -  in June 2011. It is soft tissue cancer although it usually has some tumor evident at the skin level. I want to share my Canadian experience in case it can help inform others.

The pathologist who read my biopsy recommended I be referred to a dermatologist in Vancouver. I was absolutely blessed because the doctor in Vancouver had experience with DFSP and was a Mohs surgeon. However, he had never had a patient with DFSP in such an unusual location - which made surgery in the clinic impossible.

Mohs surgery (named after the doctor that invented the process) involves taking the tumor out and then doing tissue analysis during the operation to ensure that all the cancer cells are gone. Patients stay awake while layers of tissue are removed until clear margins are achieved. Mohs surgery removes less healthy tissue while making sure the unhealthy tissue is all gone.

The Mohs surgeon in Vancouver encouraged me to do further research and referred me to the BC Cancer Agency. I read numerous research articles and summarized their findings. I found out that Mohs surgery has a 99% success rate (no recurrence after 5 years). I also found out that wide excision (taking 3 - 5 cm of tissues all around every part of this tumor including it's tentacles) has a 60 - 87% success rate (after 5 years). I also found out the recommended protocol in the United States has become Mohs surgery in the last three years. Gleevec, a relatively new cancer treatment, is also recommended as it can shrink the tumor and keep any missed cancer cells from multiplying after surgery.

The next step seemed clear to me! I started looking for Mohs surgeon who worked in a hospital operating room setting. There is no one in Canada. Then I looked for a Mohs surgeon allowed to bring his/her team into an operating room in Canada. I needed an operating room because  the location and depth of the tumor. Again no luck.

When I met with the oncology surgeon at BC Cancer Clinic he described the wide excision he would do. Imagine for a minute. 3-5 cm. It is similar to 3 to 5 of your fingers. The tumor was under my arm. Look at your shoulder. Do you have an extra 3 - 5 cms to lose? I am a writer and I travel. There was likely that I would lose the function of one arm. Not only would this impact on my life it would also impact on my work. Now itf that was the only option then better alive without the use of one arm, than dead. I get that.

But, when I asked him about Mohs surgery he said wide excision has proven to be just as successful. I know from the research that that statement is not based on fact. Another oncology surgeon I spoke with at Princess Margaret said if Mohs surgery was worth knowing about they would already know about it. Think about that statement. The only things worth knowing is what is already known? That is a scary statement from a health professional.

As patients we must be informed. There are too many cancers for the medical professionals to know about all of them. DFSP is such a rare cancer very few people know how to treat it. For example my local surgeon was dismayed that he couldn't remove it. He said, "I don't know why you are going to Vancouver. I could do that surgery." At the time I wondered myself. What neither of us knew then is that DFSP gets more aggressive AFTER surgery. If any cancer cells are missed (and this is  a soft tissue cancer- hard to find those cells ) and DFSP recurs, it is nasty and hard to overcome. If the local surgeon had done the surgery (even with the best of intentions) I would likely be dead within a year.

If you or a loved one is diagnosed with DFSP, please do your research. There is no hurry to rush into a decision about treatment. This is a slow growing tumor (grows over many years) until it is not. You will likely have the time to do your research and make the best decision for you and your case.

If you have questions I would be happy to tell you what I've learned so far and get you started on your own research.

PS Since I first posted this I have been referred to a facebook site with hundreds of members... http://dfsp - DERMATOFIBROSARCOMA PROTUBERANS... I recommend you also join that group as well as posting here.

Here are the articles I found helpful in making a case for Mohs surgery rather than wide excision in my case:

http://www.ncbi.nlm.nih.gov/pubmed/14983497

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2989807/

http://www.ncbi.nlm.nih.gov/pubmed/18520205

http://www.ncbi.nlm.nih.gov/pubmed/14983497

http://www.ncbi.nlm.nih.gov/pubmed/8682970

http://www.ncbi.nlm.nih.gov/pubmed/9088462

http://www.ncbi.nlm.nih.gov/pubmed/15070606

http://www.ncbi.nlm.nih.gov/pubmed/18684568

http://www.wjso.com/content/8/1/48

http://www.ncbi.nlm.nih.gov/pubmed/21421145

Addition December 23, 2013

I just found out that Mohs micrographic surgery is now available in operating rooms in Alberta, Ontario and Newfoundland!! Yahoo!!! Change is coming!

Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by CancerConnectionTeam on Nov 21, 2011 7:44 pm

Hi WestCoast,

Thank you for sharing your story with our community.  It sounds like you have worked hard to advocate for yourself and become informed about your rare form of cancer.

You may want to check out the Rare Cancer group at this link.

Best Wishes,

Sara, CancerConnection Team

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by bald5972 on Jan 4, 2012 11:34 pm

Hi there,

I am very grateful for anyone who is writing about this rare cancer as my 2 year old son was just diagnosed with this.  We have been asking for help since he was 3 months old and are greatful that we finally had an answer.  As his is noted as agreesive (fast growing) we have had 2 biopsies and are scheduled for surgery for his DFSP diagnosis.  Extremely rare, and even more so in children.

I guess my question is did you get the MOHS, we are schedule for surgery and would love to know of any information, suggestions, Dr's, or anything we can do as parents, and advocates for our son's health.

Sincerely grateful, PRB

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Jan 4, 2012 11:54 pm

Hi,
I'm so sorry to hear about your son.
A couple of questions... Have you seen a Mohs' surgeon? Does the surgery need to be done in a hospital or can it be done in a clinic? This is an important question for you to ask because research shows Mohs' surgery is 99% effective (no recurrence after 5 years) whereas regular surgery varies from 58%- around 90% effective. Plus Mohs surgery is the least invasive and has a quicker recovery time.
My prayers are with you all,

Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Jan 4, 2012 11:55 pm

PS Are you in Canada? If so, what province or territory?
Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Jan 5, 2012 12:55 am

Another question... has your son been given any drug treatments yet?

I know nothing about children and DFSP but I do know that Health Canada (and US authorities) recommend Gleevec as a treatment for DFSP pre and post surgery (see document posted on Health Canada website and search for additional information online - there is lots of information available).

I had a PET CT scan right away to see if the DFSP cells had metastasized anywhere else... they tend to go to the lungs. Have your son's lungs been checked?

An oncologist I worked with shared case histories of adult patients where the DFSP tumors disappeared using Gleevec. In my case Gleevec was used for two weeks prior to surgery and will be used for a full year after surgery. Gleevec does something to the cells... something to do with the chromosomes and DNA...

The other drug that I have been taking each week during the same time period is Octreotide. This drug prevents growth hormone... which means all growth slows down... this isn't an issue for me as an adult but I suspect would be significant for a two year old. I will only be on Octreotide for about 6 months in total.

I was also treated for four weeks following surgery with five days of 5FU (Fluorouracil) followed with two days of Vitamin C IV's... the 5 FU killed the remaining cancer cells and the Vitamin C IV's pushed them out while boosting my immune system. This is a very low dose chemotherapy that is administered for 24 hours/day through IV... as a result there was no adverse reaction other than I felt tired.

The other thing I have learned is that the BC Cancer Society did a study to find out what was most effective... the regular medical treatment for cancer, alternative treatments for cancer or a combination. Their research study, I am told, found that a combination was most effective. With this research finding in mind, I want to share some of the complementary "cancer treatments" that have been recommended to me (I have no idea about whether they would be right for your son... you would want to talk to a naturopathic oncologist ). Some can be done easily. Others are more difficult for different reasons. I will list them briefly. You can search online to find out more information about any of these:

No sugar of any kind... sugar feeds cancer and you don't want to feed cancer... so no fruit, no white bread or pastries and so on....

No iron supplements... iron feeds cancer... researchers recommend a diet free of red meat and free of anything that might have growth hormone... for example, free range chicken not regular store bought

Careseng - produced in Canada and has proven an effective part of a complete anti-cancer plan. A naturopathic doctor/naturopathic oncologist will help with this

Vitamin C IV's help support the immune system and, if I remember correctly, help to clear out dead cancer cells... see a naturopathic doctor/naturopathic oncologist to help with this

Poly MVA - one tsp three times a day (goggle polymva to find out more)... great results documented

Tian Hua Fen - Chinese herb that apparently slows down cell growth... one tsp three times a day

B17 (Laetrile) - comes from apricot pits... lots of research done over the years. Not available in Canada at this time

Gendicine - this is only available in China - it is expensive but amazing results have been documented

I had Mohs surgery three months ago. It was amazingly successful. I could move my fingers and keyboard the next day. I am currently regaining full mobility and strength for my arm (my tumor was located in my left axilla (left armpit) and have begun to swim as well as use weights under the supervision of my doctor (he specializes in sports medicine). I will have a PET CT scan in three more months to check for glucose uptake (cancer cells will suck up the glucose and sparkle... and will be seen on the PET CT). I am conscious that I have to take care of myself... forever....

When you are talking with health care practitioners remember they are there to help you AND you need to be a partner in this process... ask questions and politely demand the best care for your son and for you... you are both worth it... and the best care is given to those who politely insist on it... and, you won't know you are getting the best care unless you do your research... find a family member or a friend to help with the research... we cannot do it alone.

I hope some of this helps....
You and your son are in my prayers.

Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Jan 5, 2012 9:52 am

HI Again,
I was thinking about my  local surgeon who thought he could do the surgery.... please make sure you see an oncologist BEFORE any surgery. There is some mention in the research I've done that even a biopsy (which is necessary) can make DFSP grow faster and be more agressive. It is documented as becoming MORE agressive after surgery which is why the before and after surgery cancer care is so important. My medical team (regular medical oncologist and naturopathic oncologist) did everything they could to make sure that the remaining DFSP cells (if any) have no chance to grow and take root again. I would not let just any surgeon - no matter how good s/he is - do this surgery.
I am continuing to pray.
Blessings,

Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by bald5972 on Jan 9, 2012 9:59 pm

hi there, MY husband and i are very appreciative and overwhelmed by all the information you have provided and plan on reviewing and taking notes for my apt with oncology and the surgeon tbis week. we live close to toronto. we sincerely thankyou.

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Jan 9, 2012 10:39 pm

Hi,
I'm so glad some of what I sent along is useful. I am pleased to hear you are meeting with an oncologist and the surgeon.
One of my son's friends lives in Toronto and she had cancer as a very young child. The care she received and the support her parents received was amazing.
I am confident you will find your way to the right experts to help you.
If you want to receive copies of the research I have done to respectfully share with them  I'll send the collection of reports I found useful along with websites to you.
You all continue to be in our prayers.
Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Jan 9, 2012 10:45 pm

PS Consider taking along another adult to the appointments who can take notes (and maybe even record the session so you can transcribe the notes later).

I have a pretty good brain but I found I needed someone to do this. My brain would get hijacked with emotion. I need someone who would not get emotional because I couldn't care for them as well as for myself.

You need someone who can get the facts as there is so much to remember and you can't do it on your own. Then, afterwards (for days and days afterwards) if you are like me you will end up going back to the notes to check to see if they match your memory.

Unless you are in this place of emotional terror, I don't think one can't understand how hard it is to process all the information coming your way.

You need a team of people to help you. Find someone. If you don't have someone contact the Cancer Society for someone - there will be a volunteer somewhere who can help you... maybe my son's friend could help... let me know....
Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by mommy on Jan 12, 2012 11:03 pm

Hello.  I hope it will give you comfort to know that our 7 yr old son just had a MOHS procedure to remove his dfsp tumour from near his ankle.  He is doing well.  We are still waiting the final pathology but we have been very blessed to be working with a fantastic team of people at BC Children's.  I hope all goes well for your little boy too. 

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by Elkchaser on Jan 16, 2012 12:20 pm

Hi I was just Diognosed with DFSP last week after my biopsy came back. my lump was on my right collarbone. They are sending me to a dermatologist named Dr. John P. Arlette here in Calgary where I reside. I am a little eerie after googling this doctors reviews from his former patients. Can you google him also and tell me your honest opinion if he would be the best choice? I don't know how health care works if it would be possible for me to be covered in Vancouver?

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Jan 16, 2012 5:21 pm

Hi,

I'm sorry you have to go through this. It can be so frightening.

As you are thinking about doctors... there is more than one certified Mohs surgeon in Alberta.. check them all out... just make sure they have done the full training... if you google Dr. Cowan, Vancouver you will find a link that lists all the Mohs surgeons in Canada... they need to have been trained in the program that takes more than a weekend or two... look for American College of Mohs Micrographic Surgery and Cutaneous Oncology....

I was told and the research agrees... do your research and find the right team for you.... go see the recommended doctor and see what you think... and go see another one and another one until you find the right one for you.... it is your body and your future...

It is also important that you work with an oncologist as DFSP is cancer. You will want a comprehensive treatment plan. Ask you doctor for a referral to the cancer clinic in your province. You might also want to google naturopathic cancer care plans and see what alternative medical support will complement the support the cancer clinic will give you.

I wish you well.
Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by fedeyks on Feb 29, 2012 1:24 am

Hi Elkchaser I was diagnosed 3 days earlier than you. Have you had any progress? I am in Edmonton. Would love to exchange researched information since we are in the same province.

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by Elkchaser on Feb 29, 2012 2:43 am

Mach 19 I go in for MOHS & any recon surgery that's needed. As for any more progress or info the doctors are actually being quite vague . No MRI no PET scan just surgery date. Gess I wil know more then . How about you fedeyks?. Also my dermatologist (MOHS surgeon) has done suppose ably the 2nd most surgery,s in Canada . But truth be told I still asked my family Dr to have me referred to a oncologist as soon as the surgery is done. I don't really have a lot of faith in most Dr,s after 4 over a 6yr,s told me not to worry about it ! It's just fat or a cyst . Besides what's a 99% success rate mean ? I got a 1 in a million cancer right ? I am not very impressed with the 3month wait either , nothing like letting your mind go crazy knowing that it in you growing & wondering if you pissed it off by having the tumor cut out . Sorry for the rant just a little frustrated

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by fedeyks on Mar 2, 2012 8:34 pm

Hi Elkchaser I contacted your Dr. He is going to look at my file for a second opinion. The Mayo has my file as well, I can't have enough opinions. Am finding out my old GP ignored a letter from my previous Dermatologist in 2004. Waiting for a copy of the letter that stated to watch for DFSP. Just did some searching myself today. Not a happy camper right now. So I could have been positive for 8 years. Do you need reconstructive surgery?

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by Elkchaser on Mar 2, 2012 11:44 pm

Recon starts right after the MOHs . Mine was ignored since 2005 by 4 differnt DR,s but at least finally I had a great new GP that cared enough to go that extra step and listen to my concern . Sorry to hear that you to we're over looked seems to be the norm now. Understand the frustration @ first I wasn't to crazy with my MOHs surgeon . But it seem,s he a lot of experience & teaches it also.I will let him do his job but I am still involving a oncologist for my peace of mind. Best of luck and hope you find a team that will work with &for you . That seems to be the hardest part so far .

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Mar 4, 2012 7:41 am

fedeyks.... It is great you have the energy and support to do all this... second opinions and research can be so exhausting. It is also invigorating and life-affirming.

I was visiting with a friend and ended up talking medical stories - her husband has had some significant health issues recently - and she said she get copies of ALL test results. She reviews them, googles terms and information, and then goes to see the doctor. So smart! I've only begun and even that has been hit and miss... your experience is such a lesson for me... we have to be self advocates.

I was reading a new book out right now called The End of Illness and the author talks about the importance of tracking our own health indicators because our GP's will never have enough time to notice patterns and trends in our health. We need to notice and ask questions.... we can't trust anyone else with our health and well-being.

As I reflect on the 'end of the age of innocence' for me I think about the 'take back the night' marches... I am taking back responsibility for my good health... scary but good scary.

Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Mar 5, 2012 3:31 pm

Hi Elkchaser and Fedeyks,
Has anyone talked to you about Gleevec? It has been used successfully to reduce DFSP tumours prior to surgery and to limit them afterwards. I have a lot of research I can share. My oncologist was happy to get copies. Do you want copies? If so I think I can upload docs on this site.
Thinking of you!
Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Mar 8, 2012 11:31 am

Hi Elkchaser
I'm thinking about you. I hope you are finding ways to care for yourself. The waiting is so hard.
Blessings,

Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Mar 12, 2012 12:48 am

Hi Bald5972,
How are you doing? How is your son? You have all been in my prayers.
Blessings to you and all your loved ones.

Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by bald5972 on Mar 12, 2012 1:04 pm

Well,

it has been nothing less than a journey.  Our 2 year has had 5 surgeries in 9 weeks.  At Sick Kids they did their own approach to removal of the DFSP, they did wide margin (3cm) and sent it to pathology, the first round almost all margins were positive.  Following this we went again to have another wide margins with punch biopsies.  The result was that his removal on the back of his scalp is 10 cm margin and the punch biopsy were done at 12 cm.  Margin were all clear, so he had his skin graft done. 

As he is only two you can only imagine how we have  been able to explain this and make sense of it.  He understood and could verbalize "Dr took bumps, bumps all gone" then to get him to understand that he took skin from his bum (both sides) to cover where bumps were...... Well never underestimate what children can process, as he understood and was able to explain and verbalize back to us and tell others.

We are now in the waiting phase.  Our sons' DFSP is so rare as it is congenital, and the studies we found had sample size of 3, and no one his age or location.  So he truly is 1 in a million on so many levels. 

We understand the next year shows the highest rate of reoccurrence so we are taking it a day at a time and are so grateful for all the differing support we have received. 

If anyone is reading this and has a young child, please e-mail me, being alone (in the age range of this DFSP) has been so hard, and I am so grateful for all the people who provided positive ideas, suggestions and support.

much thanks, warm greeting from Ontario

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Mar 13, 2012 9:28 pm

Hi Bald5972,
Wow! What an account!
I am so glad your son has you... and the team you have put together...
It is amazing that he (and you) have been able to do all this... such courage and strength.... amazing... he must be a very special child... imagine being able to be such a partner with you in his health!?!
Now it is all about healing and maintaining a healthy inner terrain... one in which good health florishes and cancer cell growth is impossible... I know you will be watching and celebrating each milestone towards good health. Each day together is a blessing.
Please keep us in your celebration milestones 'loop' so we can celebrate along side you and have more hope for each one of us in this "one in a million" club...
You are all in my prayers.
Blessings!

PS I will soon celebrate 6 months clear of DFSP cancer post surgery... blessings abound...  I know... I only need to choose to look and see.... I celebrate every day of good health and happiness...
Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Mar 17, 2012 6:56 am

Hi Elkchaser,

I hope your surgery goes even better than expected and hoped for! I'll be thinking and praying for you on Monday.

A.
Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!

RE: Dermatofibrosarcoma Protuberans (DFSP)

Posted by WestCoast on Mar 19, 2012 3:53 pm

Hi Elkchaser,
You are in surgery today... chasing good health! May all go better than expected. You are in our thoughts and prayers today.
Westcoast.... After two cancers I am still....Happy, healthy and choosing JOY!