2021 Volume 67 Issue 5 Pages 329-333
Langerhans cell histiocytosis (LCH) is a disease of unknown cause in which Langerhans cells proliferate monoclonally, and it has a very low prevalence of 1-2 per million. We herein report a pediatric case of LCH originating from the eye socket. The patient was a five-year-old boy. One month earlier, swelling of the left eye socket had occurred and rapidly enlarged, so he was examined at the ophthalmology department of a local general hospital. A malignant tumor originating from the eye socket was suspected, and the patient was referred for a detailed examination and treatment. At the first visit to our hospital, the destruction of the orbital bone on the outside of the left eye was observed, and the skin covering the outer corner of the left eye was easily crushed, exposing a hemorrhagic mass. Magnetic resonance imaging showed a tumor having an unclear boundary with the lateral rectus muscle and orbital adipose tissue, and positron emission tomography-computed tomography showed hyperaccumulation, consistent with an orbital tumor. A biopsy was performed, and acidophilic atypical cells with a strong tendency to necrotize were found. Immunostaining revealed positive results for CD1a and CD163. Based on the above, a diagnosis of LCH was made. Clinically, the diagnosis was LCH localized to a single organ. Oral predonisolone was started at 60 mg/m2/day and then gradually reduced. A marked regression of the tumor with a favorable disease course was achieved.
