Tracheal paraganglioma presenting as stridor in a pediatric patient, case report and literature review

Objective: To review tracheal paragangliomas and describe the clinical presentation, radiologic findings, operative management, and histologic findings of a pediatric patient who presented with stridor refractory to traditional asthma therapy. Methods: Chart review of an 8-year-old male who presented to a tertiary care pediatric hospital and literature review of tracheal paragangliomas. Results: We present the case of an 8-year-old male who presented with new-onset of wheezing and dyspnea on exertion. He was given a new diagnosis of asthma and treated with bronchodilators that failed to improve his symptoms, which progressed over 3 months until he presented urgently with biphasic stridor. Bedside flexible laryngoscopy failed to reveal an etiology. Computed tomography (CT) imaging demonstrated 17 x 12 x 16 mm exophytic mass arising from the posterior membranous trachea with extension of the mass to the border of the thyroid gland and separate from the esophagus. Magnetic resonance imaging (MRI) angiography confirmed vascular supply from the right thyrocervical trunk and inferior thyroid artery. Rigid microlaryngoscopy revealed a friable vascular polypoid mass 2 cm distal to the vocal folds with 75% obstruction of the airway from which a small biopsy was taken. Pathology confirmed paraganglioma with neuroendocrine cells arranged in "zellballen" architecture and strong immunopositivity for chromogranin and synaptophysin in the neuroendocrine cells and S100 immunopositivity in the sustentacular cells. The patient underwent complete open resection of the tumor including three tracheal rings with primary anastomosis. Final pathology confirmed paraganglioma and negative margins. Genetic screening revealed a succinate dehydrogenase complex subunit C (SDHC) germline mutation, confirming hereditary paraganglioma/pheochromocytoma syndrome. He remains well at 3 month follow up without dyspnea or stridor. Conclussion: Tracheal paragangliomas are exceptionally rare, with 12 reported cases. This is the only pediatric case reported. In pediatric patients with persistent airway complaints, subglottic and tracheal masses and obstruction should be considered. Due to the vascularity and endotracheal component of tracheal paragangliomas, a detailed surgical plan should consider embolization, endotracheal laser photocoagulation and electrocautery, and open surgical resection. Additionally, pediatric patients benefit from a multidisciplinary approach including radiology, endocrinology, and genetic counseling.