General
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• Clonal proliferation of Langerhans cells, either isolated or part of systemic process
• Usually males, teens to twenties
• Common sites are middle ear and temporal bone
• Rarely involves labyrinth in adults (Otol Neuroto 2004;25:27)

Xray description
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• Single or multiple sharply circumscribed osteolytic lesions

Case reports
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• 5 year old boy with histiocytosis X of temporal bone (Pol Merkur Lekarski 2003;14:250)

Treatment
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• Excision (curettage) or low dose radiation therapy
• Good prognosis, cure if no recurrence within 1 year
• Chemotherapy if systemic

Micro description
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• Sheets, nests or clusters of Langerhans cells, which have moderate eosinophilic cytoplasm, bean-shaped nuclei with indentations, vesicular chromatin with small nucleoli
• Neutrophils, plasma cells, lymphocytes, variable foamy histiocytes and multinucleated giant cells
• No/mild tumor cell pleomorphism, no/rare mitotic figures

Micro images (other sites)
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H&E, CD1a

 

Various images

Cytologic images
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Temporal bone

Spine - B: Diff Quik; C: Pap (arrow at eosinophil); D: CD1a; E: H&E

Positive stains
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• Langerhans cells: S100, CD1a
• Histiocytes and multinucleated cells: CD68

Electron microscopy description
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• Birbeck granules

Differential diagnosis
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• Sinus histiocytosis with massive lymphadenopathy: CD1a negative
• Non-Hodgkin lymphoma: CD20+, S100-, CD1a-

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