Inflammatory Myofibroblastic Tumor: A Rare Case Report

Abstract

Inflammatory myofibroblastic tumors (IMTs) are rare benign tumors that can occur anywhere in the body, most commonly in the pediatric and young adult populations. The gold standard treatment is surgical resection, possibly along with chemotherapy and/or radiotherapy. IMTs have a high recurrence rate and may present with secondary symptoms, such as hemoptysis, fever, and stridor. We present a 13-year-old male patient with hemoptysis for one month who was subsequently diagnosed with an obstructing IMT of the trachea. The preoperative assessment showed the patient was not in acute distress and could protect his airway, even when lying flat. The treatment plan was discussed with the otolaryngologist, to keep the patient spontaneously breathing throughout the case. Anesthesia was induced with boluses of midazolam, remifentanil, propofol, and dexmedetomidine. Doses were adjusted as needed. Glycopyrrolate was also given to limit the patient's secretions before initiating the surgical procedure. The FiO₂ was kept under 30% as tolerated to reduce the risk of airway fire. During surgical resection, the patient was kept spontaneously breathing, and paralytics were avoided. Due to high tumor vascularity and inability to obtain hemostasis, the patient was kept intubated and on ventilation post-operatively until definitive treatment could be performed. On postoperative day 3, the patient returned to the operating room due to a worsening condition. He was found to have a partial obstruction of the right mainstem bronchus by the tumor. More of the tumor was debulked, and he remained intubated above the level of the debulked mass. The patient was then transferred to a higher acuity institution for advanced care. After the transfer, the patient underwent a carinal resection on cardiopulmonary bypass. This case provides insight into successfully sharing the airway during tracheal tumor resection, emphasizing minimizing the risk of airway fire and constant communication with the surgeon.

Keywords: general anesthesia laryngoscopy; hemoptysis; pediatric anesthesiology; pediatric surgery; tracheal tumor.

Figure 1. The bronchoscopic view of the tracheal tumor. The large size of the tumor caused the patient to be stimulated intermittently upon airway manipulation during the procedure.

Figure 2. A computed tomography image with contrast depicting an endobronchial mass seen within the distal trachea (red circle) without atelectasis or infiltration. The mass involving the proximal part of the right bronchus was moderately obliterating the tracheal lumen and measured approximately 8 x 11 x 19 millimeters.

Figure 3. A computed tomography angiographic image with intravenous contrast depicting a soft tissue mass in the trachea measuring 19 millimeters (red circle). It did not demonstrate significant arterial enhancement.