Case report: Epithelioid inflammatory myofibroblastic sarcoma treated with an ALK TKI ensartinib

Li, Mengmeng; Xing, Ruyue; Huang, Jiuyan; Shi, Chao; Wei, Chunhua; Wang, Huijuan

doi:10.3389/fonc.2023.1084456

Cited by 3 publications

(2 citation statements)

References 41 publications

(72 reference statements)

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“…Several case reports of the use of ALK-TKIs for the treatment of PRRC2B-ALK rearranged epithelioid inflammatory myofibroblastic sarcoma provide successful examples of the durable efficacy of targeted therapy. [ 49 , 50 ] In the follow-up analysis of the REGOSARC study, [ 51 ] which was included in our meta-analysis, no meaningful prognostic and predictive biomarkers for response to regorafenib treatment were found. But mutations in genes associated with angiogenesis were found in this study, suggesting the feasibility of an anti-angiogenic targeted therapy.…”

Section: Discussionmentioning

confidence: 99%

Efficacy of tyrosine kinase inhibitors in patients with advanced or metastatic sarcomas after prior chemotherapy: A meta-analysis

Li,

Liu,

Liang

et al. 2024

Medicine

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Background: Sarcoma is a heterogeneous malignancy arising from interstitial tissue. Anthracycline-based therapy is the first-line treatment recommended by guidelines for patients with locally advanced or metastatic unresectable sarcoma. Recently, targeted therapies, in particular tyrosine kinase inhibitors (TKIs), have made significant progress in the treatment of sarcoma, and their efficacy has been investigated in randomized controlled trials. The aim of this meta-analysis is to evaluate the efficacy of TKIs in patients with advanced or metastatic sarcoma who have previously received chemotherapy. Methods: We completed a meta-analysis after conducting literature searches in PubMed, Embase, and Cochrane. The single-drug, placebo-controlled, randomized controlled clinical trials of TKIs in patients with advanced or progressive sarcoma who have previously received chemotherapy are available for inclusion in the study. The observation results were objective response rate (ORR), disease control rate (DCR), progression-free survival (PFS), and overall survival (OS). The subgroup analysis was performed according to histological subtypes of sarcoma. Results: This study included 6 studies, including 1033 patients. The ORR (OR: 7.99, 95% CI: 3.62–19.61, P < .00001), DCR (OR: 2.54, 95% CI: 1.27–5.08, P = .009), PFS (HR: 0.46, 95% CI: 0.34–0.62, P < .00001), and OS (HR: 0.80, 95% CI: 0.67–0.96, P = .02) of patients treated with TKIs were better than those in the placebo group. Conclusions: In patients with advanced sarcoma, TKIs have been shown to have advantages in terms of ORR, DCR and PFS and OS. Multi-targeted TKIs may be considered as one of the second-line treatment options for sarcoma patients who have received prior chemotherapy.

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Section: Discussionmentioning

confidence: 99%

Efficacy of tyrosine kinase inhibitors in patients with advanced or metastatic sarcomas after prior chemotherapy: A meta-analysis

Li,

Liu,

Liang

et al. 2024

Medicine

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“…Based on previous case reports, ensartinib has demonstrated better efficacy in the treatment of brain metastatic non-small cell lung cancer with STRN-ALK fusion ( 32 ). 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 ).…”

Section: Discussionmentioning

confidence: 99%

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

Li,

Zheng,

et al. 2023

Front. Oncol.

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Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a highly aggressive malignant subtype of inflammatory myofibroblastoma (IMT) associated with poor prognosis. IMT can occur in various parts of the body, most frequently in the lungs, followed by the mesentery, omentum, retroperitoneum, and pelvis, among other areas; however, it is exceptionally rare in the stomach. Anaplastic lymphoma kinase (ALK) is a critical driver of lung cancer development and is currently the “gold standard” target for non-small cell lung cancer treatment. However, there are few reports on the use of ALK inhibitors for EIMS, necessitating further investigation. A male patient with postoperative inflammatory myofibroblastic sarcoma of the stomach received postoperative chemotherapy and had a stable outcome. However, a repeat CT scan performed 11 months later revealed disease progression. The patient later underwent immunohistochemistry testing that indicated ALK positivity, and next-generation sequencing revealed STRN-ALK fusion. Ensartinib 225 mg qd was administered as recommended, and the patient experienced only mild pruritus and no adverse effects such as rash. Eight months after CT follow-up, the patient’s subseptal soft tissue nodules had decreased, and the outcome was assessed as a partial response. The findings of this case report introduce a novel strategy for treating ALK-positive EIMS that utilizes ensartinib, a drug with previously demonstrated success in the treatment of ALK-positive cancer.

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A case report: Pathological complete response to neoadjuvant lorlatinib for Epithelioid inflammatory myofibroblastic sarcoma with EML4-ALK rearrangement

Zheng,

Zhao,

Ren

et al. 2024

Front. Pharmacol.

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Inflammatory myofibroblastic tumor (IMT) is a rare tumor originating from mesenchymal tissue. Epithelioid inflammatory myofibroblastic sarcoma (EIMS) represents a rare and particularly aggressive variant, associated with a worse prognosis. Almost all EIMS cases exhibits activating anaplastic lymphoma kinase (ALK) gene rearrangements, which suggests that EIMS patients may potentially benefit from treatment with ALK tyrosine kinase inhibitors (TKIs). We presented a case involving a 34-year-old woman who was diagnosed with mediastinal EIMS and had a rare echinoderm microtubule-associated protein-like 4 (EML4) -ALK fusion. Following 15 months of neoadjuvant lorlatinib treatment, the patient underwent a complete surgical resection, resulting in a pathological complete response. Given the heightened risk of postoperative recurrence associated with EIMS, the patient’s treatment plan included ongoing adjuvant therapy with lorlatinib. As of the present moment, the patient has achieved an overall survival of over 2 years with no observed tumor recurrence. Consequently, the case offers valuable clinical evidence supporting the potential benefits of neoadjuvant lorlatinib treatment for ALK-positive locally mediastinal EIMS patients, with a demonstrated tolerable safety profile.

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Case report: Epithelioid inflammatory myofibroblastic sarcoma treated with an ALK TKI ensartinib

Cited by 3 publications

References 41 publications

Efficacy of tyrosine kinase inhibitors in patients with advanced or metastatic sarcomas after prior chemotherapy: A meta-analysis

Efficacy of tyrosine kinase inhibitors in patients with advanced or metastatic sarcomas after prior chemotherapy: A meta-analysis

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

A case report: Pathological complete response to neoadjuvant lorlatinib for Epithelioid inflammatory myofibroblastic sarcoma with EML4-ALK rearrangement

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