Desmoid tumors of the head and neck: a therapeutic challenge

Head Neck. 2014 Oct;36(10):1517-26. doi: 10.1002/hed.23496. Epub 2014 Jan 13.


Desmoid tumor, or aggressive fibromatosis, is a rare, histologically benign, fibroblastic lesion that infrequently presents in the head and neck. Desmoid tumors often grow locally, invasively, and may, in rare instances, be fatal secondary to invasion into critical structures, such as airway or major vessels. The most common treatment is surgery, but desmoid tumors are characteristically associated with a high local recurrence rate after resection. Although the margin status seems to be of importance, operations that avoid function loss and esthetic disfigurement should be the primary goal. The efficacy of postoperative radiotherapy is controversial. Its potential benefit should be carefully balanced against possible radiation-induced adverse effects. Alternative treatment modalities, such as primary radiotherapy and medical treatment or a wait-and-see policy, may be preferable to mutilating surgery. Considering all the aforementioned, it seems obvious that desmoid tumors of the head and neck present a therapeutic challenge and require an individualized approach.

Keywords: aggressive fibromatosis; desmoid; desmoid tumor; head and neck.

Publication types

  • Review

MeSH terms

  • Diagnosis, Differential
  • Fibromatosis, Aggressive / diagnosis
  • Fibromatosis, Aggressive / etiology
  • Fibromatosis, Aggressive / genetics
  • Fibromatosis, Aggressive / radiotherapy
  • Fibromatosis, Aggressive / surgery
  • Fibromatosis, Aggressive / therapy*
  • Head and Neck Neoplasms / diagnosis
  • Head and Neck Neoplasms / etiology
  • Head and Neck Neoplasms / genetics
  • Head and Neck Neoplasms / radiotherapy
  • Head and Neck Neoplasms / surgery
  • Head and Neck Neoplasms / therapy*
  • Humans
  • Magnetic Resonance Imaging
  • Radiotherapy, Adjuvant
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Watchful Waiting