Dermatofibrosarcoma protuberans: (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.
The 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.
Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon soft tissue neoplasm with intermediate- to low-grade malignancy.
Dermatofibrosarcoma Protuberans: A review of its epidemiology, clinical features, diagnosis, histopathology, treatments, and prognosis.
n. A slow-growing dermal neoplasm consisting of one or more purplish nodules that tends to recur but usually does not metastasize
Background. Dermatofibrosarcoma protuberans or DFSP is a sarcoma of low malignant potential. Its importance lies in its locally aggressive behavior which may ...
Dermatofibrosarcoma protuberans ORDR lists rare diseases for information purposes only and does not guarantee that a condition is rare. Read more