Histiocytosis of head and neck in pediatric population

<b>Introduction:</b> Histiocytosis develops as a result of uncontrolled and abnormal proliferation of Langerhans cells of unknown etiology. The disease can affect any organ and cause nonspecific complaints. Manifestations range from isolated benign lesions to rapid progression of a multisystem disease.<b>Aim:</b> The objectives of this study included an analysis of pediatric cases of histiocytosis as described in the literature over the last 10 years, identification of ENT manifestations of Langerhans cell histiocytosis (LCH) in the pediatric population, and presentation of authors’ own experience and clinical observations from the treatment of LCH in children.<b>Material and methods:</b> A comprehensive literature query was conducted on the PubMed and Cochrane Library databases. The results were limited to papers printed between 2013 and 2023. The inclusion criteria comprised cases diagnosed and histopathologically confirmed as LCH, cases with symptoms involving the head and neck region in the pediatric population under 18 years of age, detailed case reports, and articles written in English.<b>Results:</b> A total of 16 articles involving 272 pediatric patients with ENT symptoms of LCH were included and evaluated in this review. Three case reports from the authors’ center were also included.<b>Conclusions:</b> LCH within the pediatric population is a rare disorder, potentially atypical in clinical presentation. LCH should be taken into account in differential diagnosis of difficult-to-treat recurrent otitis media in children. Characteristic presentation of LCH consists in numerous osteolytic bone lesions as seen in imaging studies. In most patients, the disease is characterized by a favorable prognosis.