title: Langerhans cell histiocytosis of the orbit: five clinicopathologic cases and review of the literature.
abstract: Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as unisystem (unifocal or multifocal) or multisystem disease. Orbital involvement typically manifests as a solitary lesion that carries a favorable prognosis. We describe the clinical and histologic spectrum of LCH of the orbit in our five cases. One patient exhibited multifocal unisystem disease; the other four had a localized process. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunohistochemistry. Transmission electron microscopy demonstrated characteristic intracytoplasmic Birbeck granules. We review the different ophthalmic manifestations of LCH and treatment strategies. As LCH may solely involve the orbit, treatment is based on the degree of organ involvement. LCH should included in the differential diagnosis in tumors of the ocular adnexae, especially in young children.
authors.affiliation: Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia 30322, USA.
authors.forename: Martina C
authors.initials: MC
authors.lastname: Herwig
authors.forename: Ted
authors.initials: T
authors.lastname: Wojno
authors.forename: Qing
authors.initials: Q
authors.lastname: Zhang
authors.forename: Hans E
authors.initials: HE
authors.lastname: Grossniklaus
mesh_terms: D049109:Cell Proliferation; D002648:Child; D002675:Child, Preschool; D003475:Curettage; D004804:Eosinophils / Q000473:pathology; D005260:Female; D015726:Giant Cells / Q000473:pathology; D005938:Glucocorticoids / Q000627:therapeutic use; D006646:Histiocytosis, Langerhans-Cell / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007223:Infant; D015552:Injections, Intralesional; D007801:Langerhans Cells / Q000473:pathology; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009916:Orbital Diseases / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D014057:Tomography, X-Ray Computed
chemical_list: D005938:Glucocorticoids; D008775:Methylprednisolone