Head and Neck or Intrathoracic Desmoid Tumor: When Medical Therapy Has Failed to Control Disease

Clinical scenario

This protocol applies to patients with desmoid tumor arising in the head and neck or the intrathoracic region whose disease has not been adequately controlled by an initial course of systemic medical therapy.

Prior treatment — failure condition

The preceding line involved systemic medical therapy — including agents such as nirogacestat, sorafenib, pazopanib, low-dose chemotherapy, or liposomal doxorubicin — sustained for a course of at least 6 months. Escalation to this protocol is warranted when imaging at 6 months demonstrates continued disease progression, or when tumor size stability or reduction has not been achieved.

Next step — treatment approach

Following confirmed failure of medical therapy, the protocol outlines locally directed procedural interventions aimed at achieving durable local disease control. Specific eligibility and selection criteria guide which approach is appropriate for each case.

Full options, sequencing, and clinical criteria are in the structured protocol below.

Clinical goals

Tumor non-progression on imaging and sustained local disease control.

Instant Access to Structured Evidence-Based Regimens

References

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.

DOI: 10.1001/jamaoncol.2024.1805

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