Locally Advanced Inflammatory Myofibroblastic Tumor Treated With Targeted Therapy: A Case Report and Literature
Charis Durham1, Matthew Clemons2, Alwin Alias3
1Hematology and Medical Oncology, Baylor University Medical Center, part of Baylor Scott and White Health, Dallas, USA.
Abstract:
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.
Insights
Anaplastic lymphoma kinase (ALK)-rearranged inflammatory myofibroblastic tumors show favorable responses to ALK-targeted tyrosine kinase inhibitors (TKIs). This case report highlights crizotinib
Area of Science:
- Oncology
- Molecular Pathology
Background:
- Inflammatory myofibroblastic tumors (IMTs) are characterized by genetic alterations, notably anaplastic lymphoma kinase (ALK) gene rearrangements.
- Other molecular targets in IMTs include ROS1, NTRK, and PDGFR, influencing therapeutic strategies.
Observation:
- This case report details a patient with locally advanced pulmonary IMT harboring an ALK rearrangement.
- The patient received neoadjuvant-intent crizotinib, an ALK-targeted tyrosine kinase inhibitor (TKI).
Findings:
- The patient exhibited a highly favorable response to crizotinib, leading to the decision to forgo surgery.
- A comprehensive review of published IMT cases treated with systemic therapy, including duration and outcomes, is presented.
Implications:
- ALK-targeted TKIs represent an effective treatment for advanced or metastatic ALK-rearranged IMTs.
- Further research is needed to establish optimal duration and sequencing of TKI therapy due to limited data.
