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.