Objective: To report a case of Langerhans cell histiocytosis of the temporal bone presenting with cerebrospinal fluid fistula.
Patient: A Caucasian woman presented to a tertiary care centre in Quebec, Canada, with a new onset of cerebrospinal fluid fistula. She had a significant destructive lesion of the temporal bone, and was diagnosed with Langerhans cell histiocytosis on biopsy.
Interventions: The patient underwent surgical resection with reconstruction of the posterior fossa and tegmen. She suffered a relapse less than one year after surgery, and was finally treated with chemotherapy.
Main outcome and results: The patient was free of disease at three-year follow up. No recurrence of the cerebrospinal fluid leak was observed after treatment.
Conclusion: Langerhans cell histiocytosis of the temporal bone with intra-cranial involvement is rare in adults, with only two cases previously reported. Eleven paediatric cases have been reported. To our knowledge, this patient represents the first report of cerebrospinal fluid fistula as the initial presentation of the disease.