Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment  

作　　者：PAULINA CHMIEL ALEKSANDRA SŁOWIKOWSKA ŁUKASZ BANASZEK ANNA SZUMERA-CIEĆKIEWICZ BARTŁOMIEJ SZOSTAKOWSKI MATEUSZ J.SPAŁEK TOMASZŚWITAJ PIOTR RUTKOWSKI ANNA M.CZARNECKA 

机构地区：[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

出　　处：《Oncology Research》2024年第7期1141-1162,共22页肿瘤学研究（英文）

基　　金：National Science Center 2019/35/O/NZ2/03761(AMC)。

摘　　要：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.

关 键 词：Inflammatory myofibroblastic tumor(IMT) Epithelioid inflammatory myofibroblastic sarcoma Tyrosine kinase inhibitors(TKI) Anaplastic lymphoma kinase(ALK) 

分 类 号：R7305[医药卫生—肿瘤]