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Langerhans Cell Histiocytosis of Maxillary sinus
A B Azreen1, C Y Kwan, N Prepagaren
1University of Malaya, Faculty of Medicine, 50603 Kuala Lumpur, Malaysia. areein@yahoo.com.
The Medical Journal of Malaysia
|June 18, 2013
Summary
Langerhan cell histiocytosis (LCH) is a rare pediatric disease. This case highlights rare maxillary sinus involvement in a multi-organ LCH presentation, emphasizing high mortality risks despite chemotherapy.
Area of Science:
- Pediatric Oncology
- Histiocytosis Research
- Rare Diseases
Background:
- Langerhan cell histiocytosis (LCH) is a rare clonal proliferative disorder of indeterminate dendritic cells.
- Typically affecting the pediatric population, LCH can present with single or multi-organ involvement and an unpredictable disease course.
- While head and neck region involvement is common (90%), maxillary sinus involvement is exceptionally rare.
Observation:
- A 2-year-old boy presented with multi-organ LCH.
- Affected sites included the orbit, skull, maxillary sinus, and liver.
- This presentation represents a rare manifestation of LCH, particularly the sinus involvement.
Findings:
- The patient's condition was classified as high-risk, multi-organ LCH.
- Chemotherapy is the primary treatment modality for this LCH group.
- Despite treatment advancements, high mortality rates persist in high-risk LCH cases.
Implications:
- This case underscores the importance of considering LCH in pediatric patients with unexplained multi-organ involvement, even with rare site presentations.
- Further research into novel therapeutic strategies is crucial for improving outcomes in high-risk LCH.
- Early diagnosis and aggressive management are critical for potentially improving survival rates in pediatric LCH.