Temporal Bone Langerhans Cell Histiocytosis: An Uncommon Bilateral Presentation

Langerhans cell histiocytosis (LCH) is a rare condition that presents clinically in various ways. The cause and subsequent development of LCH are idiopathic and not fully understood. This disease is mainly seen in childhood. It is rare to have bilateral temporal bone LCH as the initial presentation. LCH can affect many organs. However, the bilateral involvement of the temporal bone is very uncommon. Therefore, we believe documenting cases of this presentation can lead to a better understanding of the epidemiology and prevalence of the disease, which can contribute to its management planning. A one-year-old boy was referred to a tertiary otolaryngology clinic with bilateral postauricular swelling, hearing loss, but no tenderness or ear discharge. During the patient evaluation, a CT scan was requested to further investigate the bilateral swelling, which showed bilateral bony destructive lesions in the temporal bone area. Next, the patient was scheduled for a biopsy of this lesion under general anesthesia. A biopsy of the right mastoid confirmed the diagnosis of LCH. The patient was started on LCH IV protocol for multifocal bone lesions (MFB) with special site induction. A follow-up fluorodeoxyglucose positron emission tomography/CT (FDG PET/CT) was performed on the whole body with the impression of mild interval improvement of the temporal bones’ masses bilaterally with stable bilateral cervical lymphadenopathy. LCH is a rare pathology that requires comprehensive effort from various medical and surgical teams to reach the right diagnoses and start the patient on the best available treatment plan.