BACKGROUND
Inflammatory myofibroblastic tumor (IMT) is a rare disease with a first peak before the age of 20 years and a second one between 50 e 60 years2. It was first described in 1973 as a primary lung tumor3 and since then, both lung and multiple extra pulmonary manifestations have been reported4,5,6.
The etiology still remains unknown but probably is related to an abnormal inflammatory response due to an immunological trigger at different antigens.
A wide spectrum of clinical and biological behavior is described, ranging from benign proliferations to intermediate–locally aggressive, intermediate–rarely metastasizing and malignant tumors.
The possibility of slow progression into a sarcoma has been reported7 as metastatic spread8.The lung, soft tissues and abdomen are the most involved primary sites. Surgery represents the stand-alone treatment for IMT, with a 91% 5-year disease free survival8. Chemotherapy was considered for unresectable, multifocal or metastatic disease with a response rate of 50-60%. Radiotherapy is usually reserved for a palliative approach, alone or in combination with chemotherapy9,10. Steroids or non-steroid anti-inflammatory drugs are also been considered7.
The ALK translocations are identified in IMT, representing an oncogenic trigger; the ALK inhibitors have changed the treatment approach for unresectable/metastatic and/or recurrent lesions, improving the prognosis and overall survival1.
The endobronchial or endotracheal localization is extremely rare but with a challenging approach considering the efficacy of focal treatment.
We reported on an endotracheal IMT case with a never yet reported TRAF3-ALK fusion transcript, and a brief review of published cases.