Management of Desmoid Tumors

Area of Science:

• Oncology
• Surgical Oncology
• Radiology

Background:

• Desmoid tumors (also known as aggressive fibromatosis) are rare, locally aggressive neoplasms.
• Their unpredictable behavior necessitates careful management strategies.

Purpose of the Study:

• To review the evolving management landscape for desmoid tumors.
• To highlight diagnostic accuracy, therapeutic options, and emerging treatment paradigms.

Main Methods:

• This is a review article, synthesizing current literature and clinical data.
• Focus on diagnostic criteria, surgical and radiation therapy roles, interventional radiology techniques, and systemic therapies.

Main Results:

• Management has shifted towards watchful waiting for most patients.
• Nirogacestat (a gamma-secretase inhibitor) is FDA-approved; other systemic therapies are under investigation.
• Local therapies (surgery, radiation, radiology-guided techniques) are reserved for specific indications.
• Improved understanding of desmoid tumor biology drives evolving treatment strategies.

Conclusions:

• A multidisciplinary approach is crucial for optimizing desmoid tumor patient care.
• Systemic therapies are increasingly important for refractory or unresectable disease.
• Active surveillance is the preferred initial strategy for many desmoid tumor patients.