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Dermatofibrosarcoma Protuberans
What is dermatofibrosarcoma protuberans?
Dermatofibrosarcoma protuberans (DFSP) is a slow growing tumor,
usually found on the trunk and proximal extremities of young to middle-aged
adults. Although it rarely metastasizes it can be locally aggressive.
It has a high rate of recurrence. The tumors can be solitary or multiple
nodules, erythematous to skin colored, and ranging from 0.5 cm to more than 10
cm.
Most cases of DFSP are associated with a specific
chromosomal reciprocal translocation, t(17;22)(q22;q13). This
results in the fusion of two genes: collagen type 1 (COL1A1) and
platelet derived growth factor (PDGF).
With what can dermatofibrosarcoma protuberans be confused?
Clinically, DFSP can resemble a keloid
or a dermatofibroma.
How is dermatofibrosarcoma protuberans diagnosed?
DFSP is diagnosed by biopsy. On histopathology, DFSP is a
dermal tumor, usually extending into the subcuticular fat. It has interwoven
bundles of spindle cells in a storiform pattern. Atypical cells are only rarely
obseved. On immunohistochemistry, DFSP stains CD34 positive and usually
factor XIIIa negative, distinguishing it from a
dermatofibroma.
How is dermatofibrosarcoma protuberans treated?
Surgical excision is required.
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What is the prognosis for dermatofibrosarcoma protuberans?
DFSP has a high rate of local recurrence, even after
surgery. The recurrence rate may be reduced with the use of
Moh's micrographic surgery.
DFSP rarely metastasizes.
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Date created 04/01/2008
Last updated
01/03/2009
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