Langerhans cell histiocytosis

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

Author: Nat Pernick, M.D.

Topic Completed: 1 November 2013

Revised: 28 August 2019

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

PubMed Search: Langerhans cell histiocytosis [title] ear

Page views in 2019: 271

Page views in 2020 to date: 27

Table of Contents

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

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

Microscopic (histologic) images

Images hosted on other servers:

H&E, CD1a

Cytology images

Images hosted on other servers:

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

Differential diagnosis

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