FS-DFSP (Fibrosarcomatous dermatofibrosarcoma): Anyone else?

I have a March 6th appointment. Yesterday though I met with my hematologist/oncologist for some advice. I see her for Factor V Leiden.

I have my Ph.D. from the University of Illinois. This is a research degree. so I have done extensive research about dermatofibrosarcoma. It turns out that this is caused my the translocation 17;22. This information was just amended a few days ago to my pathology report.

I will update as new information comes along.

thank you for moving me here,

Susan

Wish you well. Most people do have time to stop and plan without issue.
The other suggestion, is to keep an eye on the Clinical Trials.Gov website, as from time to time new research is undertaken. I have not looked at this site for some time, so I cannot say if there is anything running at present. However, solid tumour research is going on somewhere in the world all the time and options may arise.
Also, the manufacturers of Gleevec also offer programs to some categories of Patient assisting with cost. This may be regionally limited and not available in some countries. Look for Novartis Patient Assistance Foundation.

The mention of Little Rock, Arkansas made me smile. One of the greatest Professional supporters of DFSP Support Groups, Pathologist A/Prof Jerad Gardner was at University of Arkansas Little Rock until just a few years ago. Jerad is now at Geisinger in Danville PA. Wonderful man, a rising superstar of Dermatopathology in Bone and Soft Tissue tumours. I dare say Dr Burnett would probably know him.

Thank you, thank you, thank you !!!!!!!!!!!!!!!!
Susan

Thank you for this information. I will ask him.

Susan

@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. 🙏💕👍

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?

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.