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Cholesteatoma Masquerading as Recurrent Langerhans Cell Histiocytosis
Adrien Gendre1, Marine Dréno2, Julie Boyer3
1Department of Otolaryngology, Head and Neck Surgery, Nantes University Hospital, Nantes, France.
The Journal of International Advanced Otology
|January 31, 2023
Summary
Langerhans cell histiocytosis can affect the temporal bone, mimicking other conditions. In cases of recurrent ear symptoms, consider acquired cholesteatoma, not just disease recurrence.
Area of Science:
- Otolaryngology
- Pediatric Oncology
- Histiocytosis
Background:
- Langerhans cell histiocytosis (LCH) is a rare disorder with significant temporal bone involvement (up to 60%).
- Otological symptoms in LCH are often non-specific, complicating differential diagnosis.
- Differential diagnoses include infections, benign (e.g., cholesteatoma), and malignant skull base lesions.
Observation:
- A 14-year-old patient with a history of multifocal LCH presented with chronic ear discharge.
- Initial suspicion of LCH recurrence prompted consideration of systemic treatment.
- Imaging and surgical exploration revealed a secondary acquired cholesteatoma originating from a dehiscent posterior ear canal wall.
Findings:
- Surgical exploration confirmed acquired cholesteatoma, distinct from LCH recurrence.
- The cholesteatoma was successfully removed via a canal wall down procedure.
- This case highlights the importance of considering cholesteatoma in LCH patients with recurrent otological symptoms.
Implications:
- Accurate diagnosis is crucial for appropriate management of temporal bone lesions in LCH patients.
- Acquired cholesteatoma should be a key consideration in the differential diagnosis of recurrent otological issues in this population.
- This case underscores the need for a comprehensive diagnostic approach, including surgical exploration when indicated.
