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Camila Bragança Xavier



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    P84 - Targeted Therapy - Clinically Focused - ALK (ID 261)

    • Event: WCLC 2020
    • Type: Posters
    • Track: Targeted Therapy - Clinically Focused
    • Presentations: 1
    • Moderators:
    • Coordinates: 1/28/2021, 00:00 - 00:00, ePoster Hall
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      P84.12 - Complete Response to Alectinib Following Crizotinib in an ALK-Rearranged Metastatic Inflammatory Myofibroblastic Tumor (ID 2956)

      00:00 - 00:00  |  Presenting Author(s): Camila Bragança Xavier

      • Abstract
      • Slides

      Introduction

      Inflammatory myofibroblastic tumor (IMT) is a rare entity. The lung is the most frequent primary site, accounting for up to 1% of all pulmonary lesions. The most common metastatic sites are the lungs and brain. There is scant evidence regarding the management of metastatic disease. Approximately 50–70% of IMT harbor an Anaplastic Lymphoma Kinase (ALK) gene rearrangement.

      Methods

      Herein we report a case of an ALK-rearranged metastatic IMT exhibiting complete extra-cranial response after target treatment with crizotinib. Central nervous system (CNS) initially persisted as a sanctuary site, achieving complete response after local therapies and systemic treatment with second-generation ALK inhibitor alectinib.

      Results

      A previously healthy 20-year-old male patient reported chest pain and shortness of breath in July 2018. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) revealed a hypermetabolic paramediastinal lesion in pulmonary right lower lobe and a CNS hypermetabolic lesion in the posterior parietal lobe. Core biopsy of the pulmonary lesion was consistent with IMT. Immunohistochemistry (IHC) stained positive for ALK 5DF3. Patient was started on crizotinib 250 mg twice daily, achieving a complete pulmonary response in May 2019 (Figure 1).

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      Despite pulmonary response, magnetic resonance imaging (MRI) confirmed persistent CNS disease. Craniotomy followed by hypofractionated stereotactic radiotherapy to the surgical cavity was performed. Patient continued on crizotinib until December 2019, when multifocal CNS disease progression occurred. Treatment was changed to alectinib 600 mg twice daily, leading to a complete response in May 2020 (Figure 2).

      figure 4.png

      Conclusion

      CNS often represents the unique site of disease progression in patients receiving crizotinib, supporting a primary pharmacokinetic failure due to poor blood-brain barrier penetration. As illustrated by our case, local therapies followed by maintenance crizotinib may prolong progression-free survival (PFS). After crizotinib failure and/or among patients with CNS metastatic disease, alectinib demonstrates a high response rate and longer PFS, which is also consistent with our observational findings.

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