Desmoid-Type Fibromatosis: Evolving Treatment Standards
Marco Fiore1, Andrea MacNeill2, Alessandro Gronchi1
1Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian, 1, Milan 20133, Italy.
Surgical Oncology Clinics of North America
|September 4, 2016
Summary
Desmoid-type fibromatosis, a rare neoplasm, is shifting from surgery to nonoperative management. New biological insights are guiding improved treatment strategies and therapeutic targets for this condition.
Area of Science:
- Oncology
- Neoplasms
Background:
- Desmoid-type fibromatosis is a rare, nonmetastasizing neoplasm with unpredictable behavior.
- Historically managed with surgery, it presents challenges like high recurrence rates and poor functional outcomes.
Purpose of the Study:
- To explore recent biological discoveries in desmoid-type fibromatosis.
- To identify prognostic/predictive features and novel therapeutic targets.
- To outline an optimized treatment algorithm for desmoid-type fibromatosis.
Main Methods:
- Review of current understanding of desmoid-type fibromatosis biology.
- Analysis of historical and evolving treatment modalities.
- Development of a proposed treatment algorithm.
Main Results:
- Understanding desmoid-type fibromatosis biology is advancing.
- A shift towards nonoperative management is evident.
- Medical treatments include NSAIDs, hormone therapy, chemotherapy, and targeted agents.
Conclusions:
- Novel therapeutic targets and prognostic markers are emerging for desmoid-type fibromatosis.
- Nonoperative management is increasingly favored over surgery.
- A structured treatment algorithm aims to optimize patient care.
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