Successful Treatment of Temporal Bone Langerhans Cell Histiocytosis with Oral Steroids: A Case Report

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease; treatment recommendations are based on organ involvement and the extent of the disease. We present a patient with a solitary lesion of LCH with BRAF-V600K mutation which was successfully managed with systemic steroids and followed up for a long time. A 39-year-old man presented with persistent right otitis media with effusion despite treatment. LCH was diagnosed by punch biopsy, and temporal computed tomography (CT) showed an invasive, enhancing soft tissue mass extending from the malleolus to the epitympanic recess. Due to the patient’s young age and the localization of the lesion, radiation and surgery were not considered. The patient was treated with systemic methylprednisolone. The patient’s symptoms resolved after treatment and the tumor regressed completely. The case had a good prognosis in the 5-year period. This is the first report of a LCH case with the BRAF-V600K mutation, highlighting a unique aspect of the disease. The successful therapy of the temporal bone LCH with systemic steroids alone, underscores the potential effect of this treatment for the management of unifocal lesions in critical locations. Further studies and case reports are needed to expand our understanding of LCH and explore optimal therapeutic approaches for different mutation subtypes.

Keywords

• Langerhans cell histiocytosis
• BRAF-V600K
• Temporal bone
• Steroid

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