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Extra-abdominal desmoid fibromatosis
1Department of Pathology, University of Texas M.D. Anderson Cancer Center, Houston 77030.
The Annals of Otology, Rhinology, and Laryngology
|April 1, 1994
Summary
Desmoid fibromatosis is not benign, often occurring in the head and neck. Surgical excision has high recurrence rates, highlighting the need for effective management strategies for this fibroblastic tumor.
Area of Science:
- Oncology
- Pathology
- Surgical Oncology
Background:
- Desmoid fibromatosis presents a significant clinical challenge due to its aggressive nature.
- This condition frequently affects the head and neck region, with a predilection for the supraclavicular area.
- Intraosseous (desmoplastic fibroma) and soft tissue variants are recognized.
Purpose of the Study:
- To underscore the non-benign biological behavior of desmoid fibromatosis.
- To highlight the high rates of recurrence and persistence following surgical intervention.
- To differentiate desmoid fibromatosis from other fibroblastic proliferative disorders like keloids.
Main Methods:
- Review of clinical presentations and outcomes of desmoid fibromatosis.
- Analysis of recurrence and persistence rates associated with surgical excision.
- Comparative pathology of desmoid fibromatosis, keloids, and proliferative scars.
Main Results:
- Desmoid fibromatosis demonstrates high recurrence and persistence rates, exceeding 50% after intralesional or marginal excision.
- The head and neck, particularly the supraclavicular region, are common sites for this tumor.
- Keloids and proliferative scars, though persistent, are distinct reactive conditions.
Conclusions:
- Desmoid fibromatosis requires careful consideration due to its aggressive potential and high recurrence rates.
- Surgical management strategies must account for the high likelihood of disease persistence.
- Accurate differentiation from reactive fibroblastic conditions is crucial for appropriate patient management.