Abstract
<title>Abstract</title> <p> <bold>Objective:</bold> To investigate the clinical features, treatment, and prognosis of Langerhans cell histiocytosis (LCH) in children presenting with initial oral and maxillofacial manifestations. <bold>Methods:</bold> A retrospective analysis was conducted on 26 children diagnosed with LCH via mandibular surgery at Beijing Children's Hospital (2018–2021) who presented with initial oral and maxillofacial symptoms. Demographics, clinical features, imaging, disease classification (single-system [SS], multisystem without [MS-RO−] or with risk organ involvement [MS-RO+]), treatment, and outcomes were analyzed. <bold>Results:</bold> The cohort included 17 males and 9 females (ratio 1.89:1), with a median age of 45.9 months (range 6–136 months). All patients presented with jaw swelling, and 8 (30.8%) reported pain. The mandible was involved in all cases, most frequently the mandibular angle (73.1%). Disease classification was SS in 69.3%, MS-RO− in 11.5%, and MS-RO+ in 19.2%. At a median follow-up of 68 months, no deaths occurred. Disease progression during chemotherapy varied significantly by disease type: SS 22.2%, MS-RO− 66.7%, and MS-RO+ 80% (P=0.033). Permanent bone defects occurred in 27.3% of patients undergoing curettage, whereas none of those undergoing biopsy alone developed such defects. <bold>Conclusion:</bold> Children with oral and maxillofacial LCH have a favorable prognosis, with disease progression during chemotherapy determined by systemic involvement rather than local surgery. Simple biopsy should be preferred for diagnosis to minimize impact on jaw development. </p>