The Journal of International
Advanced Otology
Case Report

Cholesteatoma Masquerading as Recurrent Langerhans Cell Histiocytosis

1.

Department of Otolaryngology, Head and Neck Surgery, Nantes University Hospital, Nantes, France

2.

Department of Otolaryngology, Head and Neck Surgery, Clinique Confluent, Nantes, France

3.

Department of Pediatric Otolaryngology, Nantes University Hospital, Nantes, France

4.

Department of Pediatric Oncology and Immunology, Nantes University Hospital, Nantes, France

J Int Adv Otol 2023; 19: 70-73
DOI: 10.5152/iao.2023.22716
Read: 1785 Downloads: 481 Published: 01 January 2023

Langerhans cell histiocytosis is a rare condition affecting the temporal bone in up to 60% of cases. Symptoms are non-specific and the differential diagnosis includes infection, benign lesions such as cholesteatoma, and malignant lesions of the skull base. Here, we report the case of a 14-yearold child referred with chronic ear discharge, and background of multifocal Langerhans cell histiocytosis 9 years prior. Recurrence of Langerhans cell histiocytosis was initially suspected and systemic treatment was considered. Further imaging workup and surgical exploration of the mastoid showed a secondary acquired cholesteatoma arising from a dehiscent posterior ear canal wall. Surgical removal of the cholesteatoma was performed with a canal wall down procedure. We review the presentation and management of temporal bone Langerhans cell histiocytosis. We recommend that cholesteatoma should be considered in case of recurrence of otological symptoms in patients with a background of Langerhans cell histiocytosis.

Cite this article as: Gendre A, Dréno M, Boyer J, Thomas C, Michel G. Cholesteatoma masquerading as recurrent langerhans cell histiocytosis. J Int Adv Otol. 2023;19(1):70-73.

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