Inflammatory myofibroblastic tumor of the upper airways harboring a new
TRAF3-ALK fusion transcript.
Abstract
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
golden 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
therapies1. 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 before unknown TRAF3-ALK
fusion transcript.