Abstract: Objective? To investigate the clinical characteristics and treatment of juvenile xanthogranuloma ( JXG) presenting as a solitary tracheal mass in children. Methods? Clinical data of a patient admitted to the first affiliated hospital of Xiamen University with tracheal JXG in March 2019 was retrospectively analyzed, and related literatures were reviewed . Results? A 20-month-old girl, had a course of more than 2 months with repeated stridor and inspiratory dyspnea. She was treated with inhalation budesonide and alternating albuterol in addition to a systemic steroid, but relief was minimal. Evaluation of the subglottic area by bronchoscopy identified a light-yellow mass with a diameter of about 0 . 8 cm on the lateral wall of the trachea below the glottis which resulted in 60% of the trachea obstructed. The tumor was removed by laser under the microlaryngoscope, and locally treated with CO2 cryotherapy. Histopathological and immunohistochemical examination confirmed the diagnosis of JXG involvement of the trachea. Follow up for 2 months showed no recurrence. With the “juvenile xanthogranuloma, children” as keywords, the CNKI, Wangfang digital knowledge service platform and PubMed were searched for relevant articles from the establishment to August 2020 . Eight cases of children with JXG of trachea or larynx were reported. Including this case, all the 9 patients had recurrent wheezing as the main manifestation, and the conventional anti-infection and antiasthmatic treatment did not have a good effect. Some of the cases were surgically excised, some were conservatively treated after tracheotomy, waiting for the natural degeneration of the lesion, and most of them had a good prognosis. Conclusion? JXG of trachea or larynx is rarely seen in children. JXG can remit spontaneously in some cases, therefore surgical treatment was only required when severe airway obstruction occurs. Laser resection of tumor combined with CO2 cryosurgery can not only relieve airway obstruction, but also reduce the formation of local granulation tissue.

Key words: juvenile xanthogranuloma;? trachea;? child