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Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment

PAULINA CHMIEL1,2, ALEKSANDRA SłOWIKOWSKA1,2, ŁUKASZ BANASZEK1,2, ANNA SZUMERA-CIEćKIEWICZ3, BARTłOMIEJ SZOSTAKOWSKI1, MATEUSZ J. SPAłEK1,4,*, TOMASZ ŚWITAJ1, PIOTR RUTKOWSKI1, ANNA M. CZARNECKA1

1 Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
2 Faculty of Medicine, Medical University of Warsaw, Warsaw, 02-091, Poland
3 Department of Pathology, Maria Sklodowska Curie National Research Institute of Oncology, Warsaw, 02-781, Poland
4 Department of Radiotherapy, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, 02-781, Poland

* Corresponding Author: MATEUSZ J. SPAłEK. Email: email

(This article belongs to the Special Issue: Novel Targets and Biomarkers in Solid Tumors)

Oncology Research 2024, 32(7), 1141-1162. https://doi.org/10.32604/or.2024.050350

Abstract

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with intermediate malignancy characterized by a propensity for recurrence but a low metastatic rate. Diagnostic challenges arise from the diverse pathological presentation, variable symptomatology, and lack of different imaging features. However, IMT is identified by the fusion of the anaplastic lymphoma kinase (ALK) gene, which is present in approximately 70% of cases, with various fusion partners, including ran-binding protein 2 (RANBP2), which allows confirmation of the diagnosis. While surgery is the preferred approach for localized tumors, the optimal long-term treatment for advanced or metastatic disease is difficult to define. Targeted therapies are crucial for achieving sustained response to treatment within the context of genetic alteration in IMT. Crizotinib, an ALK tyrosine kinase inhibitor (TKI), was officially approved by the US Food and Drug Administration (FDA) in 2020 to treat IMT with ALK rearrangement. However, most patients face resistance and disease progression, requiring consideration of sequential treatments. Combining radiotherapy with targeted therapy appears to be beneficial in this indication. Early promising results have also been achieved with immunotherapy, indicating potential for combined therapy approaches. However, defined recommendations are still lacking. This review analyzes the available research on IMT, including genetic disorders and their impact on the course of the disease, data on the latest targeted therapy regimens and the possibility of developing immunotherapy in this indication, as well as summarizing general knowledge about prognostic and predictive factors, also in terms of resistance to systemic therapy.

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APA Style
CHMIEL, P., SłOWIKOWSKA, A., BANASZEK, Ł., SZUMERA-CIEćKIEWICZ, A., SZOSTAKOWSKI, B. et al. (2024). Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment. Oncology Research, 32(7), 1141-1162. https://doi.org/10.32604/or.2024.050350
Vancouver Style
CHMIEL P, SłOWIKOWSKA A, BANASZEK Ł, SZUMERA-CIEćKIEWICZ A, SZOSTAKOWSKI B, SPAłEK MJ, et al. Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment. Oncol Res. 2024;32(7):1141-1162 https://doi.org/10.32604/or.2024.050350
IEEE Style
P. CHMIEL et al., "Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment," Oncol. Res., vol. 32, no. 7, pp. 1141-1162. 2024. https://doi.org/10.32604/or.2024.050350



cc This work is licensed under a Creative Commons Attribution 4.0 International License , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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