Choroidal Langerhans' cell histiocytosis

Abstract

ABSTRACT.Purpose: To report a patient with choroidal Langerhans' cell histiocytosis. Methods: A solitary tumor was found in the left eye of a 49-year-old male who had no definite history of systemic disorders, but had observed visual disturbances for a period of 1 month. Ultrasonography, fluorescein angiography, and indocyanine green angiography were performed and the eyeball was enucleated. We prepared the specimen for microscopic examinations. Results: Fluorescein angiographic findings of the lesion were mottled hyperfluorescence in the arteriovenous phase and strong hyperfluorescence in the late phase. Hypofluorescence in both early and late phases showed on indocyanine green angiogram. The lesion of choroid was widely infiltrated by histiocytes, though no extraocular invasion was found. Immunohistochemical studies including S-100 and CD 68 staining revealed characteristic features of Langerhans' cell histiocytosis. Electron microscopic examination of the histiocytes showed histiocytosis X body (Birbeck granule) in the cytoplasm and indented nucleus. Conclusion: We consider that this is a case of choroidal Langerhans' cell histiocytosis with no evidence of systemic lesions.

Langerhans cell histiocytosis of the uvea with neovascular glaucoma: Diagnosis by fine-needle aspiration biopsy and management with intraocular bevacizumab and brachytherapy

“…14,16 Finally, solitary choroidal LCH without systemic involvement was documented in a single case of a 49-year-old man. 17 Tsai and colleagues 16 reported a case similar to ours in which the patient noted photophobia and was found to have uveitis and an iris nodule. Our case is unique as it is the first to be confirmed by needle biopsy and controlled by brachytherapy.…”

Intraocular Langerhans cell histiocytosis in a neonate resulting in bilateral loss of vision

“…Interestingly, in two of the aforementioned reports of intraocular LCH, there was no evidence of systemic lesions despite the severe ophthalmological involvement 11,12, and the ocular LCH lesion reported by Angell et al occurred after clinical remission of systemic disease 10. Another interesting feature of the reported cases is that a significant proportion of intraocular LCH was found in adult patients 9,11,12.…”

“…Intraocular involvement is rare. A few case reports have described infiltration of the choroid, iris, and optic nerve and its sheath 4–15. It has to be remarked that the level of diagnostic certainty of these reports varies, since determination of S‐100 protein, Birbeck granules or CD1a antigens has not been universally done.…”

“…In LCH, almost any organ of the body can be involved, but some tissues, such as bone, are more frequently affected. Although bony involvement of the orbit is relatively frequent in multi‐system LCH 3, direct involvement of intraocular structures is rare with few such cases reported in the literature 4–15. We describe the case of a neonate with clinically evident intraocular LCH, in whom early and aggressive treatment resulted in remission of the disease, but nonetheless, the disease led to long‐term ophthalmological sequelae including complete bilateral loss of vision.…”

Choroidal Neovascular Membrane Formation and Retinochoroidopathy in a Patient with Systemic Langerhans Cell Histiocytosis: A Case Report and Review of the Literature

“…Diffuse infiltration of ocular tissues by proliferating histiocytes in adults is rare and only one case has been demonstrated to date in a patient with aggressive multisystemic LCH [14]. Intraocular involvement as the single manifestation of LCH is extremely rare and only 2 cases have been reported thus far [2, 15]. Interestingly, both cases presented in adults as a solitary choroidal mass.…”