Progress on orbital Langerhans cell histiocytosis
10.3760/cma.j.issn.1673-4408.2025.05.001
- VernacularTitle:眼眶型朗格汉斯细胞组织细胞增生症的研究进展
- Author:
Chenxin ZHOU
1
;
Yunze ZHAO
;
Tianyou WANG
;
Zhigang LI
;
Rui ZHANG
Author Information
1. 国家儿童医学中心 首都医科大学附属北京儿童医院血液病中心 100045
- Keywords:
Langerhans cell histiocytosis;
Orbital Langerhans cell histiocytosis;
Central nervous system-Langerhans cell histiocytosis
- From:
International Journal of Pediatrics
2025;52(5):289-294
- CountryChina
- Language:Chinese
-
Abstract:
Langerhans cell histiocytosis（LCH）is an inflammatory myeloid neoplasm characterized by aberrant differentiation or proliferation of mononuclear phagocytes. Orbital involvement，termed orbital LCH（OLCH），typically presents with periorbital masses，eyelid edema，and localized osteolytic lesions. Besides，optic nerve involvement may cause diplopia，visual impairment and so on，significantly impairing quality of life. Central nervous system-Langerhans cell histiocytosis（CNS-LCH），including central diabetes insipidus and neurodegeneration，may cause irreversible sequelae such as diabetes insipidus，progressive tremor，and ataxia，severely impacting prognosis. Current management of OLCH and its association with CNS-LCH remain controversial.The Histiocyte Society considers OLCH to be one of the risk factors for CNS-LCH，and therefore should receive 6 months of systemic chemotherapy to prevent sequelae. However，conflicting evidence suggests an unclear relationship between OLCH and CNS-LCH progression，with studies demonstrating favorable outcomes in isolated orbital cases treated through localized approaches（surgical excision，curettage，or intralesional corticosteroid injection）.Patients with unifocal OLCH exhibit favorable prognosis and potential for spontaneous resolution，and may could spare from systemic chemotherapy to avoid related adverse effects.
