Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans
Updated: 07/28/2020
© Jun Wang, MD, PhD

General features
  • Low to intermediate grade malignancy
  • Rare, usually adults 20 - 40 years, more common in blacks
  • Locally aggressive, low rate of metastasis (only after repeated failures at local control)
  • May progress to fibrosarcoma or undifferentiated pleomorphic sarcoma
Clinical presentations
Pathogenesis
  • Fusion of PDGF-beta with collagen 1 alpha 1 gene causing overexpression of PDGFB
  • Abnormal activation of PDGF receptor by overexpressed PDGFB
Key morphological features
Genetic abnormalities
  • t(17;22)(q22;q13.1): COL1A1-PDGFB
Markers
Treatment
  • Excision with wide margin of subcutaneous fat
  • Tyrosine kinase inhibitor: Imatinib

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