Table of Contents
Definition / general | Radiology description | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Cytology images | Positive stains | Electron microscopy description | Differential diagnosisCite this page: Pernick N. Langerhans cell histiocytosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/earLCH.html. Accessed December 8th, 2019.
Definition / general
- 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)
Radiology description
- Single or multiple sharply circumscribed osteolytic lesions
Case reports
- 5 year old boy with histiocytosis X of temporal bone (Pol Merkur Lekarski 2003;14:250)
Treatment
- Excision (curettage) or low dose radiation therapy
- Good prognosis, cure if no recurrence within one year
- Chemotherapy if systemic
Microscopic (histologic) description
- 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
Cytology images
Electron microscopy description
- Birbeck granules
Differential diagnosis
- Sinus histiocytosis with massive lymphadenopathy: CD1a negative
- Non-Hodgkin lymphoma: CD20+, S100-, CD1a-
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