This protocol addresses patients with desmoid tumor arising in the extremity, girdles, or chest wall who have completed a first course of treatment and in whom disease has not been adequately controlled.
Initial management may have included cryoablation or a medical therapy such as nirogacestat, sorafenib, pazopanib, low-dose chemotherapy (methotrexate and/or vinorelbine, or liposomal doxorubicin) administered for at least 6 months. Escalation to this protocol is indicated when the first line did not achieve absence of disease progression on imaging at 6 months, tumor non-progression at 12 months after cryoablation, or improved pain and functional status.
Patients who progress on medical treatment or surveillance may require a more direct interventional approach targeting local disease. The full protocol details which options apply, under what conditions each is appropriate, and age-specific considerations that govern certain modalities — that information is available in the complete regimen.
The primary objectives are tumor non-progression on imaging and local disease control.
DOI: 10.1001/jamaoncol.2024.1805
Cryoablation has been reported in case series to be an effective alternative treatment for small and moderately sized extra-abdominal DT.
Patients who progress on medical treatment or surveillance may require surgery.
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.
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