FS-DFSP (Fibrosarcomatous dermatofibrosarcoma): Anyone else?

You know it's funny I made the same comment when oncology first reached out to me. (Maybe i should go buy a lottery ticket). I had a 3cm tumor on my forehead that had finally grown big enough to decide that I should have this thing removed. Went in to a general surgeon to have it removed. He says we will send it to pathology "just in case". It came back as FS-DFSP. After two months of things seemingly moving at a snails pace I finally go in for some punch biopsies tomorrow and a ring MOHS procedure on 6-2-26. After the dermatologist creates the margins I am going back to have another surgery to remove the rest of the cancerous tissue. My question is, has anyone else had to go through two different procedures? Everything that i can find says that MOHS is the preferred treatment.

@citizendeveloper how is your treatment going?

@susangourdlady

Hi, I am in a similar situation where the notion of 7 weeks of daily radiation feels like it might be worse than the actual curative effect. I have a chronic condition called ME/CFS and the last time I exerted myself that much, I was left bedbound for 5 years.

When you spoke to your hematologist/onc., how aware were they of your situation, and was it you or them who brought up gleevec?

@cmiller1122, welcome. To help you connect with other members, I moved your post about dermatofibrosarcoma protuberans (DFSP) to this existing discussion:
- FS-DFSP (Fibrosarcomatous dermatofibrosarcoma): Anyone else?https://connect.mayoclinic.org/discussion/fs-dfsp/

Here you can connect with @citizendeveloper @biceto @karlman @susangourdlady @lala1989 @sarab74 and others who have experience with DFSP.

@cmiller1122, have you completed the diagnostic pathology now? What is your treatment plan? How are you doing?

Rare diagnosis of fibrosarcoma ex DFSP has anyone heard of this?Newly diagnosed with further pathology testing currently being done. Has anyone else heard of this fibrosarcoma ex DFSP.

I was just diagnosed with dsfp-fs as well. I had my surgery and received the news that my margins are clear. I meet with my oncologist next week. My tumor was on my lower abdomen and I have a wound vac for another 5 weeks while I wait for my reconstruction surgery with skin graft. I'm a very healthy 53 year old woman- I don't drink or smoke & so this diagnosis is very shocking!

Good morning! This is my first time to try to connect with others who have experience with DFSP, and I am a little nervous! My tumor looked like a long oval shape not unlike a gel capsule in which you would take a medication. It's located in the crease between my right thigh and the pubic area...really fun...not! It appeared sometime in the beginning of this year, 2025; I showed it to my dermatologist in April when I had something (benign) removed from my collarbone. He said it looked like a cyst and looked fine. By May, it had grown a bit and become hard and dark in shape. Although I called for an appointment right away as I became alarmed, I didn't get one until July 24, when it's behavior was not that of a cyst. So, biopsied then came the news 2 weeks later after genetic testing at a university here in Northern California.

My Mohs surgery is Sept 2, so less than 2 weeks away and it seems they are expecting a good outcome.

Here's my question -- just a few minutes ago I experienced an "aching" feeling in that area. Is that normal? Also, so for those who have had this surgery, and understanding that everyone's experience is unique, what kind of time-frame might I expect? I realize it's such a general question, but it's the first time I've had the chance to ask it?

Many thanks in advance!

DFSP typically is one or more palpable lumps surroundwd by infiltrating extensions a little like a root system on a plant. These may not be visible to scans and not visible to the human eye. Pathology is the most appropriate way of detecting if the surrounding surgical margins are clear or not.
An experienced Pathologist with Bone and soft tissue specific experience is required.

As for tumours elsewhere in the body, this is extremely rare. I know a lot of DFSP patients around the world and only a handful have multiple separate primary tumours. A childhood SYNDROME ADA SCID is responsible for some of those cases. Other tumour sites can occur on metastatic disease, again very rare cases. Lastly, there are a couple of adults I know of whom have had more that one primary tumour unrelated. Again, amazingly rare.

Odds are statistically along the lines of:
DFSP 2 TO 5 CASES PER MILLION POPULATION PER YEAR.
DFSP WITH FS, less than 10 to 15 % of that 4 to 5 cases.

Multiple primary DFSP, I have contact with more than a thousand diagnosed persons over 8 odd years and o ly know of a couple of cases of ADA SCID related and a couple of cases of adults with multiple primary tumours.

Because of the growth habit of DFSP, particularly where FS is involved, the (WLE) WIDE LOCAL EXCISION, surgery will be larger than you expect and the repair my well involve a skin flap or skin graft closure.

Most important to deal with an experienced team for DFSP.

WISH YOU WELL.

I sure do know what it is like to have so many questions about this. For me the first tumor got pretty large like the size of a chicken's egg. The others trailed off of the big one with some minor separation between them all. They were the size generally of peas. sometimes though the smaller ones would enlarge or coalesce into another large one the size of a big grape. I would be pretty sure that a CT scan or an MRI would show you if there were multiple tumors.

These tumors are usually found on the torso or arms and legs. One study I read said 29 worldwide were found in the vulvar area. For us it will be very important to have regular dermatological appointments to check for new, emerging tumors which could be in the same general area or other parts of the body.

Have you been able to see the pathology report from your tumor? That would perhaps be helpful in generating questions for your oncological appointment.

Waiting is so very hard, isn't it?

Please keep us updated on your experiences going forward. We all learn so much from each other. When is your appointment with your oncologist?

@biceto Thank you SO much for your valuable feedback on treatment considerations. I’m located in South Florida/US and your guidance is really helpful as I prepare for oncology appointment. This may be a strange question but do you know how I would know if I have multiple tumors? My diagnosis was made after a single tumor removal. In exploring the ‘mass’ prior to diagnosis, I had CT & MRI scans of the area. Of course, I’m wondering if it’s a single tumor or just the one that was large enough to be visible? Would additional tumors show up on these types of scans or are there other scans/tests such as PET scans or blood test needed to better understand the scope. One last question, do you know if the tumors are typically in same general area or are they found in various parts of the body? Please forgive me as i’m a ‘first’ for my current physicians who can’t really answer a lot of my questions as I’m awaiting my oncology appointments, who I’m sure will have a better understanding & help guide me. The waiting is difficult & brings more & more questions. Again, thank you for taking the time to share your experience & suggestions. 🙏💕👍