Dermatofibrosarcoma protuberans definition - medical term
(DFSP) A type of skin tumor that begins
as a hard nodule and grows slowly. DFSP is usually found in the dermis of the limbs
or trunk of the body. (The dermis is the inner layer of the two main layers of tissue
that make up the skin.) DFSP can grow and invade surrounding tissues but typically
it does not metastasize (spread) to other more distant parts of the body.
T he cytogenetic hallmark of DFSP is an extra ring chromosome containing material
from chromosomes 17 and 22, or, less frequently, a translocation between chromosomes
17 and 22. These chromosome rearrangements in DFSP fuse the COL1A1 gene on chromosome
17 to the PDGFB gene on chromosome 22. COL1A1 is type 1 alpha-1 chain of collagen
while PDGFB is platelet-derived growth factor receptor B.
Gleevec (imatinib), a targeted-gene drug which was developed to treat chronic
myeloid leukemia (CML), is reportedly effective in slowing the growth of DFSP in
most cases. Gleevec acts in this regard by inhibiting the PDGFB receptor.
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