Abstract :
Purpoe
To explore pecialty-related perception and treatment trategie in Langerhan cell hitiocytoi (LCH) of the orbit.
Deign
A perpective.
Method
We reviewed the reported ophthalmic experience with unifocal LCH of the orbit, analyzed current oncologic clinical trial protocol, and provided a brief ummary of contemporary knowledge and theory of LCH pathogenei.
Reult
Ophthalmic literature indicate that unifocal LCH of the orbit i uually reponive to local intervention. Current international oncologic protocol identify orbital LCH a a “central nervou ytem–rik” leion (at rik for delayed-onet diabete inipidu) and mandate a 6-month coure of chemotherapy. Analyi ugget that the latter trategy i baed on cae of orbital involvement in multifocal and multiytem dieae. The pathologic Langerhan cell continue to define and unite the LCH variant, but cytokine activation of that cell may be an earlier pathogenetic determinant. Depite a common cellular mediator, LCH may be a heterogeneou proce, with everity related to varied “uptream” trigger event.
Concluion
Treatment perpective in LCH are influenced by diimilar patient encounter and varied interpretation of the baic dieae proce. Pending documentation of linkage between unifocal orbital LCH and diabete inipidu, we recommend local intervention, with ytemic treatment reerved for incomplete repone or local reactivation or the appearance of leion elewhere. LCH undercore the need for cloe interaction between pecialit with interecting clinical interet
