Case Report: Efficacy of ensartinib treatment in pulmonary inflammatory myofibroblastic tumor with a rare GCC2-ALK fusion

Abstract

BackgroundInflammatory myofibroblastic tumors (IMTs) are rare with distal metastasis. Approximately 50% of patients have anaplastic lymphoma kinase (ALK) fusion. Patients with non-small cell lung cancer with ALK fusion are usually highly sensitive to ALK tyrosine kinase inhibitors (TKIs), but the application of TKI in IMT needs further exploration.Case presentationA 66-year-old man was diagnosed with IMT with bone metastasis, cT4N0M1c, IVB stage. Immunohistochemistry results showed that he was ALK positive, and next-generation sequencing revealed GCC2-ALK fusion in the IMT. The patient was administered first-line ensartinib 225-mg QD, which targeted GCC2-ALK fusion, and denosumab 120-mg Q4w anti-bone metastasis therapy. The patient developed a grade III rash, and the ensartinib dose was reduced to 125 mg QD; consequently, he achieved a partial response (PR), and the side effects significantly reduced. Computed tomography results showed that the patient maintained PR after 7 months of follow-up, and he was still in a state of progression-free survival without obvious side effects after 11 months of follow-up.ConclusionTo our knowledge, this is the first case of the GCC2-ALK fusion type in IMT and the first report showing that the use of ensartinib as a TKI in IMT has clinical benefits.

Case Report: Clinical response to anaplastic lymphoma kinase inhibitor-based targeted therapy in uterine inflammatory myofibroblastic tumor harboring ALK-IGFBP5 fusion

“…In another case, a patient with metastatic lung IMT with TPM3-ALK rearrangement obviously responded to ceritinib after progression on crizotinib (25). Further, a 66-year-old man with pulmonary IMT presenting with GCC2-ALK fusion was administered ensartinib therapy and achieved a PR (26), which provided a reference for patients who were crizotinib-resistant. In addition, we also observed a significant decrease in NLR during the treatment process in addition to CT scans, which may provide a new reference index for efficacy evaluation.…”

“…In another case, a patient with metastatic lung IMT with TPM3-ALK rearrangement obviously responded to ceritinib after progression on crizotinib ( 25 ). Further, a 66-year-old man with pulmonary IMT presenting with GCC2-ALK fusion was administered ensartinib therapy and achieved a PR ( 26 ), which provided a reference for patients who were crizotinib-resistant.…”

Case Report: Ensartinib for gastric epithelioid inflammatory myofibrosarcoma with STRN-ALK fusion

“…In the treatment of epithelioid inflammatory myofibroblastoma with RANBP 2-ALK fusion with ensartinib, a PR was observed in patients after 4 months, and CT scans showed partial tumor shrinkage ( 33 ). An article reported the effective treatment of a GCC 2-ALK fusion case with ensartinib, in which the patient maintained PR after 7 months of follow-up and remained in progression-free survival after 11 months of follow-up ( 34 ). A case report of effective treatment with ensartinib in a patient carrying the acquired resistance mutation ALk l1171N lung adenocarcinoma, It successfully solved the problem of ALK resistance ( 35 ).…”