A case of Langerhans cell histiocytosis arising in the temporal bone following trismus

Abstract

Langerhans’ cell histiocytosis （LCH） is a rare disease caused by monoclonal proliferation of Langerhans’ cells. We report a case of LCH arising in the temporal bone with trismus.
The patient was an 8-year-old girl who was referred to our department because of trismus. Contrast-enhanced computed tomography （CT） and magnetic resonance （MR） imaging revealed a neoplastic lesion with bone destruction in the left temporal bone and zygomatic bone. Positron emission tomography-CT revealed the accumulation of fluoro-deoxy glucose in the left temporal region and multiple lymph nodes. We performed a biopsy of the lesion under general anesthesia and obtained a histopathologic diagnosis of LCH. Based on these findings, multi-system LCH was clinically diagnosed.
She was treated with multi-drug chemotherapy for 54 weeks after initial diagnosis and obtained a complete remission. She has shown no evidence of disease for 3 years after the initial treatment.