Desmoid-type fibromatosis: toward a holistic management
Nicolas Penel1,2,3, Bernd Kasper4, Winette T A van Der Graaf5,6
1Medical Oncology Department.
Current Opinion in Oncology
|May 11, 2021
Summary
Desmoid-type fibromatosis management is challenging. Recent findings show kinase inhibitors are effective, and patient quality of life is crucial in treatment decisions.
Area of Science:
- Oncology
- Dermatology
- Genetics
Background:
- Desmoid-type fibromatosis is a rare, locally aggressive fibroblastic tumor.
- Its management presents significant treatment challenges.
- Recent research has elucidated key molecular pathways involved in its progression.
Purpose of the Study:
- To review recent data on the management of desmoid-type fibromatosis.
- To explore emerging therapeutic targets and updated guidelines.
- To highlight the importance of patient-reported outcomes.
Main Methods:
- Literature review of recent studies on desmoid-type fibromatosis management.
- Analysis of new data on molecular mechanisms.
- Inclusion of recently published international management guidelines.
Main Results:
- Kinases and ɣ-secretase play roles in desmoid-type fibromatosis proliferation and invasiveness.
- Multikinase inhibitors (sorafenib, pazopanib) show proven activity.
- A ɣ-secretase inhibitor is emerging as a potential treatment.
- New guidelines incorporate patient perspectives and quality of life.
Conclusions:
- Active surveillance with MRI is the first-line approach.
- Site-specific, stepwise management is recommended for progressive disease.
- Risk-benefit analysis and patient-centered care, including HRQoL, are essential.
- Multikinase inhibitors offer efficacy but require careful tolerability assessment.
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