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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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Published in:Children Vol. 8; no. 6; pp. 1 - 8
Main Authors: Di Ruscio, Valentina, Mastronuzzi, Angela, Russo, Ida, Neri, Marianna, Stracuzzi, Alessandra, Giovannoni, Isabella, Tropiano, Maria Luisa, De Ioris, Maria Antonietta, Milano, Giuseppe Maria
Format: Journal Article
Published: MDPI Jun2021
Online Access:View this record in EBSCOhost
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      dt: Jun2021
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      pub: MDPI
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        10.3390/children8060505
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        atl: Inflammatory Myofibroblastic Tumor of the Upper Airways Harboring a New TRAF3-ALK Fusion Transcript.
      aug:
        au:
          Di Ruscio, Valentina
          Mastronuzzi, Angela
          Russo, Ida
          Neri, Marianna
          Stracuzzi, Alessandra
          Giovannoni, Isabella
          Tropiano, Maria Luisa
          De Ioris, Maria Antonietta
          Milano, Giuseppe Maria
        affil: Department of Hematology/Oncology, Cell and Gene Therapy, Bambino Gesù Children’s Hospital, IRCCS, Piazza Sant’Onofrio 4, 00165 Rome, Italy.
      sug:
      ab: 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 ALK 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 TRAF3-ALK fusion transcript.
      pubtype: Academic Journal
      doctype: Journal Article
      ougenre: Article
    language: English
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