Inflammatory myofibroblastic tumor: molecular landscape, targeted therapeutics, and remaining challenges

被引:47
作者
Mahajan, Priya [1 ,2 ]
Casanova, Michela [3 ]
Ferrari, Andrea [3 ]
Fordham, Ashleigh [4 ]
Trahair, Toby [4 ,5 ,6 ]
Venkatramani, Rajkumar [1 ,2 ]
机构
[1] Baylor Coll Med, Texas Childrens Canc Ctr, Dept Pediat, 6701 Fannin St,Suite 1510, Houston, TX 77030 USA
[2] Baylor Coll Med, Texas Childrens Hematol Ctr, Dept Pediat, 6701 Fannin St,Suite 1510, Houston, TX 77030 USA
[3] Fdn IRCCS Ist Nazl Tumori, Pediat Oncol Unit, Milan, Italy
[4] UNSW Sydney New South Wales, Lowy Canc Res Ctr C25, Childrens Canc Inst, Kensington, NSW, Australia
[5] Sydney Childrens Hosp, Kids Canc Ctr, Randwick, NSW, Australia
[6] UNSW Med, Sch Womens & Childrens Hlth, Kensington, NSW, Australia
基金
英国医学研究理事会;
关键词
Inflammatory myofibroblastic tumor; rare tumor; pediatrics; molecular profiling; targeted therapy; crizotinib; ALK inhibition; ANAPLASTIC LYMPHOMA-KINASE; PLASMA-CELL GRANULOMA; ALK EXPRESSION; GENE REARRANGEMENT; RANBP2; GENE; FUSION; INHIBITOR; ROS1; PSEUDOTUMORS; CRIZOTINIB;
D O I
10.1016/j.currproblcancer.2021.100768
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor of intermediate malignant potential that predominantly affects children, adolescents and young adults. IMT has a predilection for the lung, abdomen, pelvis, and retroperitoneum, however, can affect any part of the body. IMT is typically localized, and multifocal or metastatic disease is uncommon. Complete surgical resection is the treatment of choice when feasible. There is no established standard of care for unresectable and advanced IMT. Approximately half of IMTs harbor anaplastic lymphoma kinase (ALK) gene rearrangements, and fusions involving ROS1, PDGFR beta, RET and NTRK have also been described. Given the molecular landscape of IMT, management of these tumors has evolved to include tyrosine kinase inhibitors and novel targeted therapeutics. This review highlights the molecular characteristics, evolution of targeted therapies and the remaining challenges in the management of IMT. (C) 2021 Elsevier Inc. All rights reserved.
引用
收藏
页数:15
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