Choroidal Mass in a Patient with Undiagnosed Pulmonary Langerhans' Cell Histiocytosis

• Published in: Ocular immunology and inflammation 2024-07, Vol.ahead-of-print (ahead-of-print), p.1-6
• Main Authors: Kalra, Gagan, Fu, Roxana, Medina Mendez, Carlos A., Errera, Marie-Hélène, Waxman, Evan L.
• Format: Article
• Language: English
• Subjects: Adult; Antigens, CD1 - metabolism; Biopsy; Choroid Diseases - diagnosis; Choroid Diseases - drug therapy; Choroid Neoplasms - diagnosis; Choroid Neoplasms - drug therapy; Choroidal mass; Diagnosis, Differential; Female; fluorescein angiography; Fluorescein Angiography - methods; Glucocorticoids - therapeutic use; Histiocytosis, Langerhans-Cell - complications; Histiocytosis, Langerhans-Cell - diagnosis; Histiocytosis, Langerhans-Cell - drug therapy; Humans; Langerhan's cell histiocytosis; Lung Diseases - diagnosis; Lung Diseases - drug therapy; optical coherence tomography; Prednisolone - therapeutic use; Tomography, Optical Coherence - methods; Tomography, X-Ray Computed; Ultrasonography; Vinblastine - therapeutic use
• ISSN: 0927-3948; 1744-5078; 1744-5078
• DOI: 10.1080/09273948.2023.2192284

Langerhan cell histiocytosis (LCH), although not a common cause, should be kept in the differential diagnosis for a patient that presents with a choroidal mass. A 28-year-old female presented with a 4-day history of vision loss and associated pain in her right eye. A dilated fundus examination revealed a deep subretinal, orange, mottled lesion with associated serous retinal detachment. Ultrasonography demonstrated a solid mass at the posterior pole. Fluorescein angiography revealed corresponding, small, punctate, hyperfluorescent areas with leakage and pooling in the late phase outlining the subretinal fluid. Optical coherence tomography confirmed the choroidal elevation and subretinal fluid. In addition to starting oral steroids for addressing the patient's acute symptoms, a metastatic workup was ordered due to the lesion's appearance. Diagnosis: Computed tomography (CT) of the chest showed nodular lesions and subsequent lung biopsy was S-100 and CD1a positive, diagnostic of Langerhan's cell histiocytosis (LCH). The patient was treated with six cycles of vinblastine and prednisolone therapy followed with a subsequent taper of steroids. Complete resolution of signs and symptoms was noted. A review of all reported cases of ophthalmic LCH with or without choroidal involvement was conducted. Diagnostic and therapeutic approaches described in these reportshave been summarized in the current manuscript. This case highlights the importance of pursuing a systemic workup in patients with uveal mass lesions. LCH should be considered in the differential of every choroidal mass.