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Year : 2020  |  Volume : 7  |  Issue : 4  |  Page : 179-183

Parapharyngeal inflammatory myofibroblastic tumor harboring fibronectin 1- ros protooncogene 1 fusion responded to crizotinib

1 Department of Oncology, National Taiwan University Hospital, Taipei, Taiwan
2 Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan
3 Department of Otolaryngology, National Taiwan University Hospital, Taipei, Taiwan

Correspondence Address:
Dr. Tom Wei-Wu Chen
Department of Oncology, National Taiwan University Hospital, No. 7, Chung Shan S. Rd., Zhongzheng Dist., Taipei City 10002
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JCRP.JCRP_25_20

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Inflammatory myofibroblastic tumor (IMT) is a rare tumor type usually arising in the thoracic or abdominal cavity. Despite its rarity, IMT commonly harbors driver gene rearrangements involving anaplastic lymphoma kinase (ALK), ROS proto-oncogene 1 (ROS1), and neurotrophic tropomyosin-related kinase. We present a rare case of the parapharyngeal IMT with convoluted diagnostic test results in determining driver gene rearrangement. The immunohistochemical stains were ALK-negative and ROS1 positive, but the result of ROS1 fluorescence in situ hybridization was equivocal. Amplicon-based targeted next-generation sequencing (NGS) did not detect any ROS1 rearrangement, but hybridization capture-based NGS revealed a rare fibronectin 1 (FN1)-ROS1 fusion. Eventually, the patient started crizotinib and had a tumor response with tolerable toxicity. This case highlights the importance of appropriate molecular testing of IMTs to guide the proper targeted therapy.

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