2024 Volume 34 Issue 1 Pages 37-43
Langerhans cell histiocytosis (LCH) is a very rare disease in which abnormal Langerhans cells proliferate. It presents with various clinical manifestations ranging from solitary to multisystem lesions, and some of the cases can be fatal. Herein, we describe a patient with LCH diagnosed with ear symptoms.
The patient was a nine-year-old female with hearing loss and otorrhea of the left ear. She was indicated to have an occupying lesion in the left temporal bone, sphenoidal bone, and clivus based on computed tomography (CT) and magnetic resonance imaging (MRI). Positron emission tomography-computed tomography (PET-CT) detected uptakes of fludeoxyglucose (FDG) in the lesions with osteolytic changes of the left temporal bone and S4 and S5 lesions of the liver. She underwent a biopsy of the tumor in the left sphenoid sinus under general anesthesia, and was diagnosed with LCH. We determined that the uptake in the liver was also a lesion due to LCH, and she underwent chemotherapy according to the LCH-12 protocol for multisystem disease with invasion of risk organs. She has maintained total remission for two years after chemotherapy without central diabetes insipidus.
