[Therapeutic options for aggressive fibromatosis]
S Altmann1, O Lenz-Scharf, W Schneider
1Klinik für Plastische, Wiederherstellungs- und Handchirurgie, Otto-von-Guericke-Universität Magdeburg. silke.altmann@medizin.uni-magdeburg.de
Handchirurgie, Mikrochirurgie, Plastische Chirurgie : Organ Der Deutschsprachigen Arbeitsgemeinschaft Fur Handchirurgie : Organ Der Deutschsprachigen Arbeitsgemeinschaft Fur Mikrochirurgie Der Peripheren Nerven Und Gefasse : Organ Der V
|April 26, 2008
Summary
Aggressive fibromatosis, or desmoid tumors, are rare, locally invasive connective tissue neoplasms. Radical surgical resection is the primary treatment, with radiotherapy for non-resectable cases to manage high recurrence rates.
Area of Science:
- Oncology
- Surgical Pathology
- Connective Tissue Neoplasms
Background:
- Aggressive fibromatosis (desmoid tumors) are rare, locally infiltrative connective tissue neoplasms.
- Classified as semi-malignant due to high recurrence and tissue destruction, but do not metastasize.
- Arise from deep musculoaponeurotic structures via monoclonal fibroblast proliferation.
Purpose of the Study:
- To evaluate the efficacy of surgical therapy for aggressive fibromatosis.
- To assess the role of adjuvant radiotherapy in managing non-resectable tumors.
- To determine optimal treatment strategies for aggressive fibromatosis based on resection outcomes.
Main Methods:
- Retrospective analysis of 9 patients (5 males, 4 females) with aggressive fibromatosis treated surgically.
- Tumors located on extremities (7), chin (1), and rectus abdominis muscle (1).
- All patients underwent radical tumor resection, followed by adjuvant radiotherapy based on resection margins.
Main Results:
- Complete tumor resection (R0) achieved in 6 cases.
- Incomplete resection (R1) occurred in 3 cases, including pelvic infiltration (2) and chin region infiltration (1).
- Adjuvant radiotherapy (25 x 2 Gy) administered postoperatively for R1 resections; chemotherapy was not required.
Conclusions:
- Aggressive fibromatosis is a semi-malignant neoplasm with high recurrence rates and destructive local growth.
- Radical surgical resection is the primary treatment of choice.
- Radiotherapy is indicated for non-resectable tumors; pharmacological treatment reserved for therapy failures.
