Langerhans Cell Histiocytosis Of The Orbit: Pediatric Case Series
Author: Shirin Hamed Azzam
Base Hospital / Institution: Baruch Padeh Medical Center,Israel
Rapid fire oral presentation
Abstract ID: 24-110
Purpose
To describe the clinical, imaging characteristics and treatment of orbital lesions of pediatric Langerhans cell histyocytosis (LCH).
Methods
Retrospective review case series of children with orbital LCH who presented at four different Centers (Tzafon, Shamir, Sheba and Moorfields eye hospital) over the past ten years. Demographic details, presenting features, imaging, histopathology, immunohistochemistry, and management outcomes were analyzed.
Results
Sixteen patients were reviewed. The mean age of presentation was 6.56±4.38 years (range: 1 to 18 years). Eyelid swelling was the most common presenting feature (13, 81%), followed by proptosis (1, 5%), eyebrow swelling (1,5%) and temple swelling (1, 5%). Imaging revealed roof osteolytic defects with superior orbital lesions in thirteen (72.2%) patients. Frontal and zygomatic bones were most commonly affected. All orbital lesions were extraconal. All included patients were diagnosed with LCH from the orbital tissue biopsy. Histopathology in all cases revealed histiocytes with numerous eosinophilic characteristics of LCH. The cells were positive for CD1a and S100 antigens. Three patients also had systemic involvement. Two patients (12.5%) had intraoperative steroids injection with curettage, and one patient (6.25%) underwent only curettage. The rest of the patients ( n=13, 81.25%) received systemic steroids and chemotherapy post-operatively. All had complete remission without any signs of recurrence, except of one case with refractory LCH.
Conclusion
LCH is a rare disorder of the orbit among the pediatric group. While the optimal treatment approach of orbital LCH remains controversial, we recommend excision and curettage where complete removal of unifocal orbital LCH is achieved. Whereas, systemic chemotherapy with steroids are recommended for incomplete removal, local recurrence of unifocal disease, and multifocal involvement.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Morris | Hartestein | Baruch Padeh Medical Center,Israel |
| Guy | Ben simon | Baruch Padeh Medical Center,Israel |
| Daphna | Landau | Baruch Padeh Medical Center,Israel |
| Fatema | Bushager | Baruch Padeh Medical Center,Israel |
| Jimmy | Uddin | Baruch Padeh Medical Center,Israel |
