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, PDGFRβ, 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.

炎性肌纤维母细胞瘤 (IMT) 是一种罕见的中等恶性潜能的间充质肿瘤，主要影响儿童、青少年和年轻人。IMT 好发于肺、腹部、骨盆和腹膜后，但可以影响身体的任何部位。IMT 通常是局部的，多灶性或转移性疾病并不常见。在可行的情况下，完整的手术切除是治疗的选择。对于不可切除的和晚期 IMT 没有既定的护理标准。大约一半的 IMT 具有间变性淋巴瘤激酶 ( ALK ) 基因重排，以及涉及ROS1、PDGFRβ、RET和NTRK 的融合也被描述过。鉴于 IMT 的分子景观，这些肿瘤的管理已经发展到包括酪氨酸激酶抑制剂和新型靶向治疗。本综述重点介绍了分子特征、靶向治疗的演变以及 IMT 管理中的剩余挑战。