2017 Volume 60 Issue 2 Pages 83-89
Langerhans' cell histiocytosis (LCH) is characterized by monoclonal proliferation and infiltration of Langerhans' cells, which are antigen-presenting cells originating from the bone marrow. The disease predominantly affects the bones, skin, lymph nodes and soft tissues. In particular, bone lesions are observed frequently, most commonly involving the temporal bone, femur and vertebral bones. Herein, we report the case of an 8-year-old boy who presented with exophthalmos caused by a posterior ethmoid mass with bone destruction. Endonasal biopsy was immediately performed and histopathology revealed the diagnosis of LCH. As imaging MRI evaluation revealed a distant lesion in the 11th vertebral bone, chemotherapeutic intervention according to the Japan LCH study group's protocol was undertaken. Until now, 10 years since treatment completion, there has been no evidence of disease recurrence. Otorhinolaryngologists should bear in mind the possibility of LCH developing in the paranasal sinuses.
