医学
间变性淋巴瘤激酶
ROS1型
克里唑蒂尼
癌症研究
酪氨酸激酶
靶向治疗
肿瘤科
内科学
病理
癌症
肺癌
腺癌
受体
恶性胸腔积液
作者
Priya Mahajan,Michela Casanova,Andrea Ferrari,Ashleigh M. Fordham,Toby N. Trahair,Rajkumar Venkatramani
标识
DOI:10.1016/j.currproblcancer.2021.100768
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, 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.
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