FS-DFSP (Fibrosarcomatous dermatofibrosarcoma): Anyone else?

I'm tagging @raeangel319 and @biceto as I believe that they have experience with FS-DFSP (fibrosarcomatous dermatofibrosarcoma).

@citizendeveloper, how was your appointment with the oncologist? What are next steps for you?

Had 3 surgeries over 5 years and only got the final fs-dfsp diagnosis after the third. I was referred to the major cancer service in Melbourne, had a wide excision removal. Got clear margins with a smallish deep margin. Follow up every 3 months incl regular scans. Follow up out to 6 monthly after 2 years then to annual. At 5 years I elected to go to a local professor to continue annual review. I am now approaching 10 yrs with no sign of recurrence. No Glivec or radiation.

Wish you well.

Thank you, Biceto!! Your message has given me great confidence in facing DFSP-FS. I am from China, and I just had Mohs surgery on the 9th of this month and am currently recovering.

I have just had 7 dermatofibrosarcomas surgically removed from my vulvar area. The gynecological surgeon did not think he was removing anything malignancy. Turns out all of them were and none of the margins were clean. I am waiting to hear about an appointment with an oncologist,

I was also just diagnosed with FS-DFSP. I’m awaiting my oncologist appointment. I located a knot on my clavicle and after several months of trying to find a physician who agreed with my concern, a very compassionate surgeon agreed to biopsy & ultimately remove for pathology although he also had no real concern prior to & after excision. His compassion & confidence gave me a lot of peace. So needless to say, the surgeon & I were both shocked with the pathology diagnosis. I can’t seem to locate much info so the feedback here is reassuring. Thank you for making me smile with your lottery comment as that’s exactly how I feel!

@susangourdlady, I moved your post to this existing discussion in the Sarcoma group:

- FS-DFSP (Fibrosarcomatous dermatofibrosarcoma): Anyone else?
https://connect.mayoclinic.org/discussion/fs-dfsp/
I did this so you can connect with others like @karlman @biceto @lala1989 and others. I'm sorry to hear that none of the margins were clean. Have you had an appointment with the oncologist to learn about next steps?

Sorry to hear of your diagnosis. I know of other women whom have been diagnosed in this area.
Are these 7 lumps close to each other, so close that they may be connected? DFSP typically has, what we diagnosed folk describe as, tentacles infiltrating the soft tissue around the main lump/s.

I am a lower back case, having had recurrences in the same location. The second had multiple lumps on it whereas the first and third were more smoothly rounded. But then the surgery to obtain clear margins was larger than expected due to needing to clear the margins of these tentacles.

Some have used Radiation and or Glivec/Gleevec to reduce the size of tumour pre surgery, others have had either treatment after surgery where clear margins were not obtained and available tissue at the site was perceived as inadequate to flap or graft over, readily.

Where are you seeking treatment?

Stay positive, seek out treatment and be comfortable with the Surgeon/Oncologist and their plans. Ask questions. Feel free to make contact if you need to chat.

Ask about Radiation Therapy, and Glivec/Gleevec and the role they may play in strategies.

Also ask if the Oncologist would consider FISH, or RT-PCR, analysis to establish the individual chromosomal mutations of your tumour. These may give insight into likely Chemotherapy or Immunotherapy options down the track.
These are expensive tests, so many Providers will not fund them for everyone. FS variant is more aggressive so more likely to be considered.
Reach out if you have questions, but include a Country and State/Province as services and options may differ between regions.

Thank you so much for this advice. I did have the extra gene work done and it is the 17;22 translocation. I have spoken with my hematologist who is also an oncologist. It was very insightful. We did talk about gleevec. I think that will be my main option. I can't have extensive surgery because I have Factor V Leiden. Extensive radiation would be too debilitating for me as I do have to run this household and property. My husband just recently had cancer surgery and will start radiation in a few weeks. He is 77 and a bit frail, so I will wait until weeks after he finishes his radiation therapy before I start anything for me.

Again, Thank you for the information.

Susan

My first tumor became the size of a chicken's egg, It did have tentacles and many more small tumors and then it seemed some of the small tumors coalesced into larger ones. There was a lot of movement of the smaller ones. All physicians thought these were sebaceous cysts and no problem for me-Ha ! & years later I find out what they really were. I am so tired.

Right now I have an appointment (March 6) with a Dr. Alexander Burnett at the University of Arkansas for Medical Sciences /cancer center in Little Rock. But yesterday I met with my hematologist/oncologist (I have Factor V Leiden). She agreed major removal of all tissue in the area was not a good option for me. Major radiation is not a good option either, But chemo may be what I end up doing. My husband (77 yrs old) is getting radiation treatment for cancer in a few weeks, I will wait until he recovers from that before I start my chemo, if chemo is what I do.

Thank you so much for you input. I do so appreciate it.
Susan