Sarcoma

Dermatofibrosarcoma Protuberans

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Dermatofibrosarcoma Protuberans

  • Definitions
  1. Dermatofibrosarcoma Protuberans
    1. Low grade fibroblastic tumor presents as a firm, nodular, Subcutaneous Mass of the trunk or proximal extremities
    2. Locally destructive but rarely metastasizes
  • Epidemiology
  1. Onset ages 20 to 59 years old
  2. No gender predominance
  3. Rare (accounts for 1% of Soft Tissue Sarcomas)
  • Findings
  1. Slow growing Subcutaneous Nodule with overlying pink Plaque
  2. Advanced lesions may ulcerate and bleed
  3. Distribution
    1. Trunk: 50 to 60%
    2. Proximal arms: 25%
    3. Head and Neck: 10 to 15%
  • Imaging
  1. Ultrasound
    1. Hypervascularity
    2. Tentacle-like projections
  • Management
  1. Refer to orthopedic oncology for core needle biopsy and wide surgical resection
  • References
  1. Achar (2022) Am Fam Physician 105(6): 602-12 [PubMed]