Surgical Management of Endobronchial Inflammatory Myofibroblastic Tumors

Abstract

Endobronchial myofibroblastic tumors are neoplasms composed of clonal populations of smooth muscle cells and a variable lymphocytic inflammatory component. They represent a challenge with respect to diagnosis, classification, and surgical resection due to their infrequent occurrence. We retrospectively reviewed our experience with patients who had myofibroblastic tumors in the major airways over a 15-year period, in order to understand the incidence, natural biology, treatment, and long-term outcome of individuals with this type of neoplasm in an endobronchial location. Between 1995 and 2010, 11 patients (9 female, 2 male) underwent surgical resection of a myofibroblastic tumor arising within the tracheobronchial tree. The mean age was 39.6 years (range, 22.3 to 53.6 years). All patients were symptomatic, with cough and dyspnea as the most common presenting complaints. Rigid bronchoscopy with endobronchial biopsy was utilized to establish the diagnosis in 9 of 11 patients. Laser-mechanical debulking was performed to relieve airway obstruction prior to operation in 10 of 11 patients. Because of wide submucosal infiltration of the neoplasms, surgical resection for complete removal was required for all individuals. Tracheal resection was performed in 3 patients, carinal resection in 1 patient, mainstem bronchial resection in 2 patients, sleeve resection in 3 patients, bilobectomy in 1 patient, and right lower lobectomy in 1 patient. Resection with tumor-free margins was accomplished in all patients. Mean tumor size was 2.3 cm (range, 1.5 to 3.5 cm). There were no operative deaths, with all patients alive and disease-free at a mean of 6.1 ± 3.7 years. Complete surgical resection of inflammatory myofibroblastic tumors presenting in a major airway is safe and leads to excellent survival for patients with this uncommon disease.