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FS-DFSP (Fibrosarcomatous dermatofibrosarcoma): Anyone else?
Sarcoma | Last Active: Feb 11 12:25am | Replies (18)Comment receiving replies
@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. 🙏💕👍
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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.