Middle ear, inner ear and temporal bone tumors - malignant
Langerhans cell histiocytosis

Topic Completed: 1 November 2013

Revised: 28 August 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Langerhans cell histiocytosis [title] ear

Nat Pernick, M.D.
Page views in 2019: 271
Page views in 2020 to date: 27
Cite this page: Pernick N. Langerhans cell histiocytosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/earLCH.html. Accessed January 26th, 2020.
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
  • 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
Microscopic (histologic) images

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H&E, CD1a

Cytology images

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

Positive stains
  • Langerhans cells: S100, CD1a
  • Histiocytes and multinucleated cells: CD68
Electron microscopy description
  • Birbeck granules
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