Pediatric inflammatory myofibroblastic tumor of the trachea: A case report and review of the literature

Abstract

Inflammatory myofibroblastic tumors (IMTs) are relatively rare entities of a distinct histologic appearance and benign clinical course. Although these lesions have been described in virtually every anatomic location, there are few documented reports with tracheal localization. We add to the short list of pediatric IMTs of the trachea, a 13-year-old boy that was referred to our pediatric respiratory unit for evaluation of his respiratory distress. In particular, we describe the clinical and pathologic features of this patient and present a review of all reported lesions in the available literature.

Adult Inflammatory Myofibroblastic Tumor of the Trachea: Case Report and Literature Review

“…32,33 The World Health Organization's defini- tion is a lesion composed of a myofibroblastic spindle cell population accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. 34 Myofibroblastic spindle cell populations account for the 70% of the total cell population in inflammatory myofibroblastic tumors, as in our patient. The symptoms are usually nonspecific, and include dyspnea, stridor, chronic cough, hemoptysis, and pleuritic chest pain.…”

“…In the small number of patients having endobronchial lesions, the clinical presentation may be acute and serious due to post-obstruction pneumonia or symptoms associated with airway obstruction. 11,34 Superior vena cava syndrome was reported as a serious associated condition in one case report of inflammatory myofibroblastic tumor of the mediastinum. 35 The etiology of inflammatory myofibroblastic tumor is unclear, but trauma to the affected region secondary to inflammation has been proposed.…”

“…Currently, due to the existence of rare cases with a more aggressive clinical picture, including local recurrence, malignant transformation, or metastasis, it was believed that inflammatory myofibroblastic tumor was a low-grade mesenchymal malignancy. 34 The findings about its recurrent chromosomal translocations involving 2p23, the anaplastic lymphoma kinase gene site, and the presence of other associated positive genetic fusions also led to the proposal that inflammatory myofibroblastic tumor was a malignant process rather than a reactive lesion. 34,[36][37][38][39][40] Applebaum et al proposed that COX2 and VEGF, as mediators of angiogenesis, might play a role in the pathogenesis and growth of inflammatory myofibroblastic tumors.…”

“…34 The findings about its recurrent chromosomal translocations involving 2p23, the anaplastic lymphoma kinase gene site, and the presence of other associated positive genetic fusions also led to the proposal that inflammatory myofibroblastic tumor was a malignant process rather than a reactive lesion. 34,[36][37][38][39][40] Applebaum et al proposed that COX2 and VEGF, as mediators of angiogenesis, might play a role in the pathogenesis and growth of inflammatory myofibroblastic tumors. 41 Surgical resection has been the most relevant treatment for inflammatory myofibroblastic tumor with tracheal involvement.…”

“…A radical surgical approach and/or adjuvant radiotherapy and chemotherapy are not indicated unless the disease is aggressive. 34 Corticosteroids and non-steroidal agents have been reported as an alternative in rare selected cases. 42 We performed intraluminal bronchoscopic removal and dilatation via rigid bronchoscopy, then corticosteroids.…”