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Inflammatory Myofibroblastic Tumor of the Upper Airways Harboring a New TRAF3-ALK Fusion Transcript

Inflammatory myofibroblastic tumor (IMT) is a rare disease that mainly involves the lung and the abdomen with an intermediate clinical course but a recurrence rate between 15-30%. Radical surgery represents the gold standard of treatment, while chemotherapy and radiotherapy are considered for unrese...

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Main Authors: Valentina Di Ruscio (Author), Angela Mastronuzzi (Author), Ida Russo (Author), Marianna Neri (Author), Alessandra Stracuzzi (Author), Isabella Giovannoni (Author), Maria Luisa Tropiano (Author), Maria Antonietta De Ioris (Author), Giuseppe Maria Milano (Author)
Format: Book
Published: MDPI AG, 2021-06-01T00:00:00Z.
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Summary:Inflammatory myofibroblastic tumor (IMT) is a rare disease that mainly involves the lung and the abdomen with an intermediate clinical course but a recurrence rate between 15-30%. Radical surgery represents the gold standard of treatment, while chemotherapy and radiotherapy are considered for unresectable lesions. The identification of <i>ALK</i> translocations in IMT opened the option for the use of target therapies. Indeed, the ALK inhibitors have changed the treatment approach for aggressive lesions, improving the prognosis. Intraluminal upper-way IMT is extremely rare and represents a medical challenge. We reported an endotracheal IMT case presenting a previously unknown <i>TRAF3-ALK</i> fusion transcript.
Item Description:10.3390/children8060505
2227-9067

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