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Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study.

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Observation is a valid treatment for extra-abdominal desmoid tumor fibromatosis (DTF), with outcomes similar to surgery. Surgery is recommended for painful lesions or those progressing during observation.

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Area of Science:

  • Oncology
  • Surgical Pathology
  • Musculoskeletal Tumors

Background:

  • Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue neoplasm.
  • Optimal management strategies for DTF remain under investigation.

Purpose of the Study:

  • Compare outcomes of DTF following various treatment modalities.
  • Identify prognostic factors for recurrence after surgical resection.
  • Determine prognostic factors for disease progression during observation.

Main Methods:

  • Retrospective multicenter study involving seven tertiary referral centers.
  • Data collected included demographics, diagnosis, tumor characteristics, clinical features, and treatment.
  • European Musculoskeletal Oncology Society (EMSOS) patronage.

Main Results:

  • 388 patients included; 66% underwent surgical excision, 18% were observed.
  • No significant differences in recurrence or progression rates between treatment groups.
  • Risk factors for recurrence post-surgery: younger age, prior recurrence, larger size.
  • Risk factors for progression during observation: shoulder girdle location, painful lesions.

Conclusions:

  • Observation is a viable approach for DTF, with recurrence rates comparable to surgical intervention.
  • Surgical intervention may be considered for lesions with dimensional progression or those causing pain.
  • Particular attention should be given to shoulder girdle lesions during observation.