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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...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8232338/ https://www.ncbi.nlm.nih.gov/pubmed/34203606 http://dx.doi.org/10.3390/children8060505 |
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author | Di Ruscio, Valentina Mastronuzzi, Angela Russo, Ida Neri, Marianna Stracuzzi, Alessandra Giovannoni, Isabella Tropiano, Maria Luisa De Ioris, Maria Antonietta Milano, Giuseppe Maria |
author_facet | Di Ruscio, Valentina Mastronuzzi, Angela Russo, Ida Neri, Marianna Stracuzzi, Alessandra Giovannoni, Isabella Tropiano, Maria Luisa De Ioris, Maria Antonietta Milano, Giuseppe Maria |
author_sort | Di Ruscio, Valentina |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-8232338 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-82323382021-06-26 Inflammatory Myofibroblastic Tumor of the Upper Airways Harboring a New TRAF3-ALK Fusion Transcript Di Ruscio, Valentina Mastronuzzi, Angela Russo, Ida Neri, Marianna Stracuzzi, Alessandra Giovannoni, Isabella Tropiano, Maria Luisa De Ioris, Maria Antonietta Milano, Giuseppe Maria Children (Basel) Case Report 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. MDPI 2021-06-15 /pmc/articles/PMC8232338/ /pubmed/34203606 http://dx.doi.org/10.3390/children8060505 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Case Report Di Ruscio, Valentina Mastronuzzi, Angela Russo, Ida Neri, Marianna Stracuzzi, Alessandra Giovannoni, Isabella Tropiano, Maria Luisa De Ioris, Maria Antonietta Milano, Giuseppe Maria Inflammatory Myofibroblastic Tumor of the Upper Airways Harboring a New TRAF3-ALK Fusion Transcript |
title | Inflammatory Myofibroblastic Tumor of the Upper Airways Harboring a New TRAF3-ALK Fusion Transcript |
title_full | Inflammatory Myofibroblastic Tumor of the Upper Airways Harboring a New TRAF3-ALK Fusion Transcript |
title_fullStr | Inflammatory Myofibroblastic Tumor of the Upper Airways Harboring a New TRAF3-ALK Fusion Transcript |
title_full_unstemmed | Inflammatory Myofibroblastic Tumor of the Upper Airways Harboring a New TRAF3-ALK Fusion Transcript |
title_short | Inflammatory Myofibroblastic Tumor of the Upper Airways Harboring a New TRAF3-ALK Fusion Transcript |
title_sort | inflammatory myofibroblastic tumor of the upper airways harboring a new traf3-alk fusion transcript |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8232338/ https://www.ncbi.nlm.nih.gov/pubmed/34203606 http://dx.doi.org/10.3390/children8060505 |
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