Desmoid Tumors: Current Perspective and Treatment.

Area of Science:

• Oncology
• Tumor Biology

Background:

• Desmoid tumors are rare, locally aggressive neoplasms.
• Lack of standardized treatment presents challenges for patient management.
• Spontaneous regression occurs in up to 20% of asymptomatic cases, supporting observation strategies.

Purpose of the Study:

• To review the evolving treatment landscape for desmoid tumors.
• To highlight recent therapeutic advancements and their clinical impact.

Main Methods:

• Literature review of desmoid tumor treatment modalities.
• Analysis of systemic therapies, including chemotherapy, hormonal therapy, NSAIDs, TKIs, and novel agents.
• Evaluation of invasive approaches like cryoablation.

Main Results:

• Traditional upfront surgery is less favored due to high recurrence and morbidity.
• Systemic therapies have advanced from older agents to targeted therapies like tyrosine kinase inhibitors.
• Gamma-secretase inhibitors demonstrate significant clinical benefit, representing a new mechanistic approach.
• Nirogacestat's recent approval marks a significant milestone in desmoid tumor treatment.

Conclusions:

• The treatment of desmoid tumors is rapidly evolving with new therapeutic options.
• Targeted therapies and novel agents are transforming patient care.
• An increasing number of new molecules are anticipated for approval, offering further hope for patients.