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Primary inflammatory myofibroblastic tumor of the trachea.

Pecoraro Y, Diso D, Anile M, Russo E, Patella M, Venuta F - Respirol Case Rep (2014)

Bottom Line: Inflammatory myofibroblastic tumors (IMTs) are rare neoplasms that can involve the airway.Recent studies have shown their malignant behavior with local recurrence and potential metastatic spread; half of the cases are associated with anaplastic lymphoma kinase gene rearrangement.We present a case of a 26-year-old woman admitted to our institution with severe respiratory failure; she was affected with primary IMT of the trachea and underwent complete surgical resection.

View Article: PubMed Central - PubMed

Affiliation: Department of Thoracic Surgery, University of Rome "Sapienza" Rome, Italy.

ABSTRACT
Inflammatory myofibroblastic tumors (IMTs) are rare neoplasms that can involve the airway. Recent studies have shown their malignant behavior with local recurrence and potential metastatic spread; half of the cases are associated with anaplastic lymphoma kinase gene rearrangement. Complete surgical resection is recommended, when feasible. We present a case of a 26-year-old woman admitted to our institution with severe respiratory failure; she was affected with primary IMT of the trachea and underwent complete surgical resection.

No MeSH data available.


Related in: MedlinePlus

At computed tomography scan: presence of a huge endobronchial lesion with stenosis of the trachea with mediastinal and subcutaneous emphysema because of the high ventilation pressures.
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fig01: At computed tomography scan: presence of a huge endobronchial lesion with stenosis of the trachea with mediastinal and subcutaneous emphysema because of the high ventilation pressures.

Mentions: A 26-year-old woman with a history of cough and wheeze treated for presumed asthma was admitted to an emergency unit of a community hospital with severe respiratory distress and stridor. At that time, she was at the seventh week of pregnancy. For the deterioration of the respiratory symptom with severe hypoxia and hypercapnia (pCO2 >120 mmHg), she was intubated. Computed tomography (CT) showed the presence of endotracheal tissue causing a tight stenosis of the airway (Fig. 1); very high ventilation pressures were required. The patient was transferred to our intensive care unit and underwent rigid bronchoscopy; multiple biopsies were obtained and most of the lesion was removed with Nd:YAG laser. Bronchoscopy confirmed the presence of an endotracheal lesion involving the distal two-thirds of the trachea for 4 cm in length, down to 2 cm from the carina. The patient was extubated 24 h after the procedure. CT was repeated and it showed a full thickness involvement of the membranous part of the trachea and the left lateral wall of the cartilaginous rings. For these reasons, definitive endoscopic treatment was not considered. Histology showed the presence of primary IMT anaplastic lymphoma kinase (ALK)+ and a tracheal resection was planned. The patient decided to voluntarily interrupt pregnancy. We subsequently performed the tracheal resection and reconstruction with an end-to-end anastomosis, removing 4.5 cm of the airway. The postoperative course was uneventful and the patient was discharged 8 days after surgery. The final histological report confirmed the diagnosis. She underwent postoperative therapy with ALK inhibitor. After 1 year, the patient is alive and well, free of disease.


Primary inflammatory myofibroblastic tumor of the trachea.

Pecoraro Y, Diso D, Anile M, Russo E, Patella M, Venuta F - Respirol Case Rep (2014)

At computed tomography scan: presence of a huge endobronchial lesion with stenosis of the trachea with mediastinal and subcutaneous emphysema because of the high ventilation pressures.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4263498&req=5

fig01: At computed tomography scan: presence of a huge endobronchial lesion with stenosis of the trachea with mediastinal and subcutaneous emphysema because of the high ventilation pressures.
Mentions: A 26-year-old woman with a history of cough and wheeze treated for presumed asthma was admitted to an emergency unit of a community hospital with severe respiratory distress and stridor. At that time, she was at the seventh week of pregnancy. For the deterioration of the respiratory symptom with severe hypoxia and hypercapnia (pCO2 >120 mmHg), she was intubated. Computed tomography (CT) showed the presence of endotracheal tissue causing a tight stenosis of the airway (Fig. 1); very high ventilation pressures were required. The patient was transferred to our intensive care unit and underwent rigid bronchoscopy; multiple biopsies were obtained and most of the lesion was removed with Nd:YAG laser. Bronchoscopy confirmed the presence of an endotracheal lesion involving the distal two-thirds of the trachea for 4 cm in length, down to 2 cm from the carina. The patient was extubated 24 h after the procedure. CT was repeated and it showed a full thickness involvement of the membranous part of the trachea and the left lateral wall of the cartilaginous rings. For these reasons, definitive endoscopic treatment was not considered. Histology showed the presence of primary IMT anaplastic lymphoma kinase (ALK)+ and a tracheal resection was planned. The patient decided to voluntarily interrupt pregnancy. We subsequently performed the tracheal resection and reconstruction with an end-to-end anastomosis, removing 4.5 cm of the airway. The postoperative course was uneventful and the patient was discharged 8 days after surgery. The final histological report confirmed the diagnosis. She underwent postoperative therapy with ALK inhibitor. After 1 year, the patient is alive and well, free of disease.

Bottom Line: Inflammatory myofibroblastic tumors (IMTs) are rare neoplasms that can involve the airway.Recent studies have shown their malignant behavior with local recurrence and potential metastatic spread; half of the cases are associated with anaplastic lymphoma kinase gene rearrangement.We present a case of a 26-year-old woman admitted to our institution with severe respiratory failure; she was affected with primary IMT of the trachea and underwent complete surgical resection.

View Article: PubMed Central - PubMed

Affiliation: Department of Thoracic Surgery, University of Rome "Sapienza" Rome, Italy.

ABSTRACT
Inflammatory myofibroblastic tumors (IMTs) are rare neoplasms that can involve the airway. Recent studies have shown their malignant behavior with local recurrence and potential metastatic spread; half of the cases are associated with anaplastic lymphoma kinase gene rearrangement. Complete surgical resection is recommended, when feasible. We present a case of a 26-year-old woman admitted to our institution with severe respiratory failure; she was affected with primary IMT of the trachea and underwent complete surgical resection.

No MeSH data available.


Related in: MedlinePlus