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Targeted therapy (ALK inhibitors)
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.
Inflammatory myofibroblastic tumors respond to ALK inhibitor therapy, as follows:
Crizotinib
Evidence (crizotinib):
For pediatric patients with measurable disease, the use of crizotinib achieved partial tumor responses in three of six patients with ALK-translocated inflammatory myofibroblastic tumors.[72]
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A case report of a patient aged 16 years with metastatic/multifocal ALK-positive inflammatory myofibroblastic tumor demonstrated a complete response and a 3-year disease-free interval with crizotinib therapy.[73]
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One study included 14 patients with inflammatory myofibroblastic tumors who were treated with crizotinib.[74][Level of evidence C3]
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One study included 14 patients with inflammatory myofibroblastic tumors who were treated with crizotinib.[74][Level of evidence C3]
Five patients had complete responses, seven had partial responses, and the remaining two had stable disease.
One study included 14 patients with inflammatory myofibroblastic tumors who were treated with crizotinib.[74][Level of evidence C3]
No patient had relapsed disease at the time the article was published.
Two adult patients with ALK-rearranged inflammatory myofibroblastic tumor achieved partial responses with crizotinib.[75][Level of evidence C3]
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An extensive review confirmed the effectiveness of crizotinib in children with inflammatory myofibroblastic tumors in various locations.[76]
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The FDA approved crizotinib for patients aged 1 year and older with unresectable, recurrent, or refractory inflammatory ALK-positive myofibroblastic tumors.
Ceritinib
Evidence (ceritinib):
Two pediatric patients enrolled in a clinical trial responded to treatment with ceritinib.[77]
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Two pediatric patients enrolled in a clinical trial responded to treatment with ceritinib.[77]
One patient had a complete response that was durable for multiple years on continuing therapy.
Two pediatric patients enrolled in a clinical trial responded to treatment with ceritinib.[77]
The other patient had a partial response when the drug was discontinued for severe liver and renal toxicity.
In a multicenter phase I study of ceritinib, 7 of 10 patients with inflammatory myofibroblastic tumor had objective responses to ceritinib.[78]
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In a phase I trial of ceritinib for adult patients who were previously treated with ALK inhibitors, one patient with inflammatory myofibroblastic tumor had a partial response.[79]
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Alectinib
A case report described the successful treatment of a patient with an inflammatory myofibroblastic tumor and a FN1::ALK gene fusion using alectinib, a second-generation ALK inhibitor.[80]
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For information about the treatment of this tumor in the lungs, see Childhood Pulmonary Inflammatory Myofibroblastic Tumors Treatment.
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Publication references
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Preserved source evidence · Not medical advice