Purpose Langerhans cell histiocytosis is a rare disease representing less than 1% of orbital tumors.
Methods We report a case of Langerhans cell histiocytosis with orbital involvement in a 3-year-old boy. He had a 3-month history of periorbital swelling of the right upper lid laterally of the left orbit (Panel A) which was increasing gradually in size. There was associated history of fever, however, no trauma, cough, headache, diabetes insipidus or weight loss. No obvious diplopia, orbital pain, proptosis, and impaired ocular movements was presented. The bump became less reddish after antibiotic medical treatment for infections（Panel B）. Computerized tomography revealed an ill-defined extraconal cellular mass involving the superolateral aspect of the left orbit with lytic bone lesion of the frontal bone, the greater wing of sphenoid bone and part of the skull base （Panel C, arrows）. A magnetic resonance image showed a mass lesion with heterogeneously gadolinium enhancement and extension to both the lateral orbit and the frontal base （Panel D）.
Results The tumor was excised using an anterior orbitotomy with an infrabrow incision for more complete curettage and resection. The tumor tissue in bone is a central destructive, lytic, aggressive-looking lesion (Panel E). Hematoxylin-eosin stain and immunohistochemical stain of biopsy slide were applied.The cytological study showed infiltrates of histiocytes derived from Langerhans cells. Immunohistochemistry with CD-68 macrophage marker , S-100 and CD1awas positive, confirming the diagnosis of Langerhans cell histiocytosis (LCH). There was no sign of the disease elsewhere on systemic investigations.
Further treatment included oral steroid and chemotherapy according to LCH protocol.
Conclusion A 2-year follow-up showed no evidence of recurrence or systemic involvement. The patient achieved complete resolution of symptoms and signs. |
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