Desmoid Tumor of the Abdominal Wall: What to Do When Active Surveillance Fails
Clinical Scenario
This protocol addresses patients with a desmoid tumor located in the abdominal wall in whom active surveillance has not been sufficient to control the lesion. Abdominal wall desmoid tumors are among the most suitable sites for intervention when continuous progression occurs, making the step beyond surveillance particularly important to manage correctly.
Why This Protocol Is Triggered
Prior approach: Active surveillance with regular clinical and imaging follow-up did not achieve its targets — specifically, stable tumor size on imaging and the absence of tumor growth at multiple consecutive follow-up appointments. Failure to hold these thresholds is the condition that escalates management to this next line.
Treatment Approach (Partial Overview)
Once surveillance is no longer adequate, intervention options for abdominal wall desmoid tumor span surgical, local ablative, and systemic medical approaches — selected according to the extent and behaviour of the lesion. The complete sequencing, eligibility criteria, and specific agents are defined in the full protocol.
Treatment Goals
- Absence of disease progression on imaging at 6 months
- Tumor size stability or reduction
- Improved pain and functional status
References
DOI: 10.1001/jamaoncol.2024.1805
- Superficial sites of disease represent the best indications for surgery in cases of continuous progression, particularly in abdominal wall DT.
- If complete resection is feasible, it can be well tolerated if the reconstruction is performed optimally, and future pregnancies are possible even with a prosthetic mesh repair.
- Cryotherapy may also be considered before surgery.
- Given its safety profile, nirogacestat may well become the first-line treatment for DTs when an active medical treatment is indicated.
- Until recently, systemic treatment options for DT consisted of only tyrosine kinase inhibitors, such as sorafenib (high quality of evidence according to GRADE) and pazopanib (very low quality of evidence according to GRADE); low-dose (very low quality of evidence according to GRADE) or conventional chemotherapeutic regimens (very low quality of evidence according to GRADE), including low-dose chemotherapy with methotrexate and/or vinorelbine administered intravenously or orally; and liposomal doxorubicin, as outlined in the 2020 consensus guideline.
- The consensus of the Desmoid Tumor Working Group was that for most of the available treatments, a course of at least 6 months is required (provided there is absence of frank progression) prior to assessing effectiveness.
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