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Langerhans cell histiocytosis of the orbit: pediatric case series
Shirin Hamed Azzam1, Salem Kassem1, Fatema Bushager2
1Ophthalmology Department, Tzafon Medical Center, Azrieli Faculty of Medicine, Bar-Ilan University, Ramat Gan, Israel.
Insights
Pediatric orbital Langerhans cell histiocytosis (LCH) presents with eyelid swelling and superior orbital osteolytic lesions. Complete remission is achievable with surgical removal and/or systemic therapy, though LCH is a rare orbital disorder.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Histopathology
Background:
- Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm.
- Orbital involvement in pediatric LCH is uncommon but can lead to significant morbidity.
- Understanding the clinical and imaging features is crucial for early diagnosis and management.
Purpose of the Study:
- To characterize the clinical presentation, imaging findings, and treatment outcomes of pediatric orbital LCH.
- To emphasize the importance of histopathological confirmation in diagnosing orbital LCH.
- To review management strategies and their effectiveness in achieving remission.
Main Methods:
- Retrospective case series analysis of pediatric patients with orbital LCH.
- Involved data collection from four centers over a ten-year period.
- Analysis included demographic details, presenting symptoms, imaging, histopathology, and treatment outcomes.
Main Results:
- Sixteen pediatric patients with orbital LCH were reviewed, with a mean age of 6.56 years.
- Eyelid swelling (81%) was the most common symptom; imaging revealed superior orbital osteolytic lesions in 72.2%.
- All patients achieved complete remission with varied treatments including surgery and systemic therapy, except for one refractory case.
Conclusions:
- Orbital LCH should be considered in pediatric patients with superior orbital osteolytic lesions.
- Histopathological diagnosis is essential for confirming LCH.
- Complete removal of unifocal orbital LCH, potentially via excision and curettage, is recommended, alongside systemic therapies if indicated.
Objective:
To describe the clinical, imaging characteristics, and treatment of pediatric orbital Langerhans cell histiocytosis (LCH).
Design:
Retrospective case series.
Participants:
Children with orbital LCH.
Methods:
Children with orbital LCH who presented to 4 different centres over the past 10 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, 1-18 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 superior orbital lesions with osteolytic defects of the orbital roof in 13 (72.2%) patients. Frontal and zygomatic bones were the most commonly affected. All the orbital lesions were extraconal. All patients in the study were diagnosed with LCH from orbital tissue biopsy. Three patients also demonstrated extra-orbital involvement. Two patients (12.5%) had intraoperative steroid injection with curettage, and 1 patient (6.25%) underwent curettage only. The remainder of the patients (n = 13, 81.25%) received systemic steroids and chemotherapy after undergoing biopsy. All patients had complete remission of disease without any signs of recurrence, except for 1 case of refractory LCH.
Conclusions:
LCH is a rare disorder of the orbit and should be considered in the differential diagnosis for osteolytic lesions involving the superior orbit among the pediatric age group. Histopathologic confirmation is mandatory. Although optimal treatment remains controversial, complete removal of unifocal orbital LCH is recommended and may be achieved through excision and curettage.
