Desmoid tumors: clinical features and treatment options for advanced disease.

  title={Desmoid tumors: clinical features and treatment options for advanced disease.},
  author={Bernd Kasper and Philipp Str{\"o}bel and Peter Hohenberger},
  journal={The oncologist},
  volume={16 5},
Desmoid tumors describe a rare monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Although histologically benign, desmoids are locally invasive and associated with a high local recurrence rate, but lack metastatic potential. On the molecular level, desmoids are characterized by mutations in the β-catenin gene, CTNNB1, or the adenomatous polyposis coli gene, APC. Proof of a CTNNB1 mutation may be useful when the pathological differential… 

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