This protocol addresses desmoid tumor of the abdominal wall in patients whose initial treatment course has not achieved disease control — specifically when imaging continues to show progression, or when tumor size and symptoms do not stabilise after a sustained first-line effort.
Desmoid tumor situated at the abdominal wall. Superficial sites such as this represent a recognised indication for intervention in cases of continuous progression.
Initial management may include surgical resection with reconstruction, cryoablation, or medical therapy with agents such as nirogacestat, sorafenib, pazopanib, low-dose chemotherapy with methotrexate and/or vinorelbine, or liposomal doxorubicin — typically sustained over a course of at least 6 months.
This next-line protocol is indicated when the first-line approach does not achieve:
Following first-line failure, the protocol centres on a radiotherapy-based strategy aimed at local disease control. Selection criteria, clinical considerations by patient age group, and the full treatment framework are detailed in the complete protocol.
Tumor non-progression on imaging and local disease control.
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.
Radiotherapy should not be used in children aged 0 to 17 years.
In young adults (aged 18-29 years), radiotherapy should be discussed individually with consideration of its late toxic effects and risk of secondary malignant neoplasms.
Postoperative radiotherapy is associated with an increased local control rate on the whole population, but it likely overtreats a large proportion of patients with indolent DT and exposes these patients to a risk of secondary malignant neoplasms; thus, it should be considered cautiously in this situation.
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