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CASE REPORT article

Front. Pharmacol.
Sec. Pharmacology of Anti-Cancer Drugs
Volume 15 - 2024 | doi: 10.3389/fphar.2024.1401428

A Case Report: Pathological Complete Response to Neoadjuvant Lorlatinib for Epithelioid Inflammatory Myofibroblastic Sarcoma with EML4-ALK Rearrangement

Provisionally accepted
Yang Zheng Yang Zheng 1,2Fanfei Zhao Fanfei Zhao 1,2Yaqian Ren Yaqian Ren 1,2Yaran Xue Yaran Xue 3Bing Yan Bing Yan 4Chun Huang Chun Huang 1,2*
  • 1 Department of Thoracic Oncology, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin, China
  • 2 Key Laboratory of Cancer Prevention and Therapy, Tianjin's Clinical Research Center for Cancer, Tianjin, China
  • 3 Department of Oncology, Tianjin Fourth Central Hospital, Tianjin, China
  • 4 Department of Precision Oncology, Tianjin Cancer Hospital Airport Hospital, Tianjin, China

The final, formatted version of the article will be published soon.

    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 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 two 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.

    Keywords: EML4-ALK, Epithelioid inflammatory myofibroblastic sarcoma, Neoadjuvant treatment, lorlatinib, Pathological complete response

    Received: 18 Mar 2024; Accepted: 22 Jul 2024.

    Copyright: © 2024 Zheng, Zhao, Ren, Xue, Yan and Huang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Chun Huang, Department of Thoracic Oncology, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin, China

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