Skin & Soft Tissue: Desmoid Tumor

General

• Fibroblastic Tumor of Fascial & Aponeurotic Connective Tissue
• Benign with No Mets
• Locally Aggressive with High Local Recurrence (30%)

Location

• Intraabdominal – Most are Unresectable at Time of Diagnosis
• Extraabdominal
  • Most Common Site: Anterior Abdominal Wall (Often Following Pregnancy or Trauma)

Associations

• Most Are Sporadic
  • Most Common Mutation: CTNNB1
• Repeated Trauma to an Area or Surgery (Seen Along a Scar)
• FAP/Gardner’s Syndrome (APC Mutation)
  • *See Oncology: Colorectal Cancer & Polyposis Syndromes

Diagnosis

• CT: Depends on Composition
  • Can be Homogenous or Heterogenous
  • Can by Hypointense or Hyperintense
• Core Needle Bx (Histology): Spindle Cells, Dense Fibrosis, High Cellularity & Low Mitotic Index

Treatment

• General Treatment: Surgical Resection
  • Can Observe if Asymptomatic & Stable in Size
• If Unresectable: XRT or Chemo (Tamoxifen & Sulindac)
• Consider Colonoscopy to Evaluate FAP