Abstract
OBJECTIVE: To explore the clinical features, diagnosis, treatment and prognosis of Langerhans cell histiocytosis (LCH) of the skull in children. METHODS: This study retrospectively summarized the clinical manifestations, treatment methods and follow-up status of children with skull LCH who were admitted to the Department of Neurosurgery of Shanghai Children's Hospital from January 2014 to June 2021. RESULTS: A total of 23 patients confirmed by histology as LCH received hospitalization treatment, including 14 males and 9 females, aged (5.76 ± 3.86) years old. The clinical manifestations were mostly incidentally discovered head masses that gradually enlarged (19 cases, 82.61%). Only 2 cases are affected by multiple systems, while the rest are affected by single systems. 9 patients were involved in multiple skull lesions, and 14 patients had local skull lesions. All patients underwent surgical intervention, with 17 patients undergoing total resection and 6 patients undergoing biopsy. 21 patients received chemotherapy after surgery. The median follow-up was 2.46 years (range 0.33-6.83 years). 21 patients had their symptoms and signs under control or even resolved, and 2 patients experienced recurrence during follow-up. The overall control rate reached 91.30%. CONCLUSION: Personalized treatment plans according to different clinical types. Regular outpatient follow-up is crucial to monitor disease recurrence and late effects.