Inflammatory Myofibroblastic Tumor Characterization and Treatment
Summary
Inflammatory myofibroblastic tumour (IMT) is a rare mesenchymal neoplasm characterised by spindle-shaped myofibroblastic cells and a chronic inflammatory infiltrate. It typically presents in the lung, abdomen and pelvis but can occur in diverse anatomical sites including the central nervous system. Histologically, IMTs exhibit variable cellularity, myxoid stroma and mixed inflammatory cells. Approximately half harbour rearrangements of the anaplastic lymphoma kinase (ALK) gene or other kinase fusions that drive proliferation. Surgical excision remains the mainstay of treatment for localised disease, although recurrence rates of up to 25% necessitate careful follow-up. In unresectable or recurrent cases, chemotherapy has been used with modest response rates. The identification of actionable kinase fusions has revolutionised management, with ALK tyrosine kinase inhibitors demonstrating high response rates. Second- and third-generation inhibitors offer options in cases of acquired resistance. Emerging strategies include immune-checkpoint blockade targeting the PD-1/PD-L1 axis, neoadjuvant and adjuvant targeted therapy for aggressive variants such as epithelioid inflammatory myofibroblastic sarcoma, and the application of gene-fusion profiling through next-generation sequencing to guide personalised regimens. Multidisciplinary approaches integrating molecular diagnostics, surgery, systemic targeted agents and novel immunotherapies are establishing new paradigms for long-term disease control and functional preservation.
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Inflammatory Myofibroblastic Tumor Characterization and Treatment publication trend
The graph below shows the total number of articles in inflammatory myofibroblastic tumor characterization and treatment across all publications each year (not limited to Nature Index journals).
Technical terms
Inflammatory myofibroblastic tumour (IMT): A neoplasm of spindle‐shaped myofibroblastic cells with a mixed inflammatory infiltrate.
Anaplastic lymphoma kinase (ALK): A receptor tyrosine kinase that undergoes oncogenic fusion, driving tumour growth in IMT.
Tyrosine kinase inhibitor (TKI): A small‐molecule drug that blocks aberrant tyrosine kinase signalling in cancer cells.
Epithelioid inflammatory myofibroblastic sarcoma (EIMS): An aggressive IMT variant with epithelioid morphology and specific ALK rearrangements.
Programmed death-1 (PD-1): An immune checkpoint receptor targeted by immunotherapies to enhance anti-tumour immune responses.
References
- Treatment of intracranial inflammatory myofibroblastic tumor with PD-L1 inhibitor and novel oncolytic adenovirus Ad-TD-nsIL12: a case report and literature review. Frontiers in Immunology (2024).
- Update of Diagnosis and Targeted Therapy for ALK+ Inflammation Myofibroblastic Tumor. Current Treatment Options in Oncology (2023).
- Inflammatory Myofibroblastic Tumour: State of the Art. Cancers (2022).
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