Langerhans cell histiocytosis of the skull in 23 children

Area of Science:

• Pediatric Neurosurgery
• Pediatric Oncology
• Histiocytosis

Background:

• Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder.
• Skull involvement in pediatric LCH presents unique diagnostic and management challenges.

Purpose of the Study:

• To investigate the clinical characteristics, diagnostic approaches, therapeutic strategies, and prognostic outcomes of pediatric skull LCH.
• To evaluate the effectiveness of current treatment protocols and the importance of long-term surveillance.

Main Methods:

• Retrospective analysis of 23 pediatric patients diagnosed with skull LCH.
• Review of clinical manifestations, imaging findings, surgical interventions, chemotherapy regimens, and follow-up data.
• Data collected from January 2014 to June 2021 at Shanghai Children's Hospital.

Main Results:

• The majority of patients (82.61%) presented with incidentally discovered, gradually enlarging head masses.
• Most cases involved single-system disease, with varied presentations of single or multiple skull lesions.
• Surgical intervention followed by chemotherapy was the primary treatment, achieving an overall disease control rate of 91.30% with a median follow-up of 2.46 years.

Conclusions:

• Tailored treatment strategies based on individual clinical presentations are vital for optimal outcomes in pediatric skull LCH.
• Consistent outpatient follow-up is critical for monitoring disease recurrence and managing potential long-term sequelae.