Current Management of Desmoid Tumors: A Review

Area of Science:

• Oncology
• Rare Cancers
• Fibroblastic Tumors

Background:

• Desmoid tumors (DT) are rare, locally aggressive fibroblastic proliferations with unpredictable behavior.
• Historically, surgery was the primary treatment, but less-invasive approaches are now favored.
• There is a need for updated global consensus guidelines on DT management.

Purpose of the Study:

• To update the 2020 global evidence-based consensus guideline for desmoid tumor management.
• To incorporate recent advancements in local therapies and novel medical agents.
• To harmonize treatment strategies among international clinicians.

Main Methods:

• Convened a consensus meeting of over 90 international sarcoma experts, patients, and advocates.
• Conducted literature searches of MEDLINE and Embase for relevant randomized clinical trials (RCTs).
• Focused on systemic therapies, local ablative therapies, and surgical indications.

Main Results:

• The 2023 guideline update includes local ablative therapies like cryotherapy and new indications for surgery.
• Incorporates data on γ-secretase inhibitors, with nirogacestat identified as the first approved drug for DT.
• Emphasizes multidisciplinary tumor boards and personalized treatment selection based on patient factors.

Conclusions:

• The therapeutic options for desmoid tumors are expanding.
• Management requires careful consideration of DT symptoms, risks, location, morbidities, and patient preferences.
• Optimizing tumor control and quality of life necessitates individualized treatment strategies.