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Durable response to the ALK inhibitor alectinib in inflammatory myofibroblastic tumor of the head and neck with a novel SQSTM1–ALK fusion: a case report

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Summary

An inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm that typically develops in the lungs and seldom in the head and neck region. It is often related to the anaplastic lymphoma kinase (ALK) fusion gene. Crizotinib, a first-generation ALK inhibitor, has been shown to have a notable response in patients with ALK-positive IMT. Here, we report the first case of a 46-year-old man with IMT harboring a novel SQSTM1–ALK fusion gene who demonstrated marked response to alectinib. The patient presented a right neck mass (5-cm diameter) that progressively enlarged and expanded to the upper mediastinum. ALK-rearranged IMT was diagnosed after complete tumor resection. Spindle cells displayed diffuse cytoplasmic staining for ALK on immunohistochemistry. A fluorescence in situ hybridization analysis revealed the translocation of a part of the ALK gene locus at chromosome 2p23. FoundationOne CDx™ assay identified an SQSTM1-ALK gene fusion. After a year, right cervical, subclavian, and mediastinal lymph node metastases, considered unresectable, developed. Notably, the patient exhibited a marked response to alectinib treatment and has sustained for 17 months following systemic therapy initiation without significant adverse events. This report highlights the possibility of alectinib being a reasonable option for advanced IMT with the SQSTM1-ALK fusion.

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Acknowledgements

The authors would like to thank Enago (www.enago.jp) for the English language review.

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Authors and Affiliations

  1. Department of Clinical Oncology, Aichi Cancer Center Hospital, 1-1 Kanokoden, Chikusa-ku, Nagoya, Aichi, 464-8681, Japan

    Kazunori Honda, Shigenori Kadowaki, Kyoko Kato & Kei Muro

  2. Department of Head and Neck Surgery, Aichi Cancer Center Hospital, Nagoya, Japan

    Nobuhiro Hanai & Yasuhisa Hasegawa

  3. Department of Pathology and Molecular Diagnostics, Aichi Cancer Center Hospital, Nagoya, Japan

    Yasushi Yatabe

Authors
  1. Kazunori Honda
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  2. Shigenori Kadowaki
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  3. Kyoko Kato
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  4. Nobuhiro Hanai
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  5. Yasuhisa Hasegawa
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  6. Yasushi Yatabe
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  7. Kei Muro
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Correspondence to Shigenori Kadowaki.

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Informed consent was obtained from the patient for the publication of this case report.

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Honda, K., Kadowaki, S., Kato, K. et al. Durable response to the ALK inhibitor alectinib in inflammatory myofibroblastic tumor of the head and neck with a novel SQSTM1–ALK fusion: a case report. Invest New Drugs 37, 791–795 (2019). https://doi.org/10.1007/s10637-019-00742-2

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  • DOI: https://doi.org/10.1007/s10637-019-00742-2

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