Locally Advanced Inflammatory Myofibroblastic Tumor Treated With Targeted Therapy: A Case Report and Literature Review.

Inflammatory myofibroblastic tumors (IMTs) are known to be associated with anaplastic lymphoma kinase (ALK) gene rearrangements. Other molecular alterations such as ROS proto-oncogene 1, receptor tyrosine kinase (ROS1), neurotrophic tyrosine receptor kinase (NTRK), and platelet-derived growth factor receptor (PDGFR) have also been identified in IMTs. Although there are no randomized controlled clinical trials comparing chemotherapy, tyrosine kinase inhibitors (TKIs), or other systemic therapies, the literature demonstrates the use of ALK-targeted TKIs as an effective strategy for the treatment of locally advanced or metastatic ALK-rearranged IMTs. This case report describes a patient with an ALK-rearranged locally advanced pulmonary IMT who was treated with neoadjuvant-intent crizotinib. The patient had a very favorable response to therapy, and surgery was declined. It is difficult to determine the duration and sequencing of TKI use in these settings as there is little published data to guide decisions. This report also includes a comprehensive compilation of published IMT cases with molecular alterations treated with systemic therapy, which also highlighted the duration of therapies and clinical outcomes.