Hematopoietic / dendritic
Langerhans cell histiocytosis

Topic Completed: 1 December 2012

Revised: 5 December 2019

Copyright: 2003-2019,, Inc.

PubMed Search: Langerhans cell histiocytosis mediastinum

Hanni Gulwani, M.B.B.S.
Page views in 2018: 219
Page views in 2019 to date: 278
Cite this page: Gulwani H. Langerhans cell histiocytosis. website. Accessed December 9th, 2019.
Definition / general
  • Rare neoplastic proliferation of Langerhans cells, a type of interdigitating dendritic cell which processes and presents antigens
  • Also called Langerhans cell granulomatosis, histiocytosis X, eosinophilic granuloma, Letterer-Siwe disease, Hand-Schüller-Christian disease
  • Usually affects bone, less commonly lymph node, skin
  • Typically thymus is unaffected
Clinical features
  • Rarely presents as thymic mass in children with myasthenia gravis, less often in adults with myasthenia gravis
Prognostic factors
  • Excellent prognosis in children
Case reports
Gross description
  • Large, often encapsulated, tan-gray, firm cut surface with bands of gray-white fibrous tissue
Gross images

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Mediastinal mass

Microscopic (histologic) description
  • Sheets of oval cells with slightly eosinophilic cytoplasm and grooved, convoluted nuclei with fine chromatin, thin nuclear membranes, up to 5 mitoses/10 HPF
  • Mixed with sheets and microabscesses of eosinophils
  • Bands of fibrous tissue, islands of residual thymus tissue
Microscopic (histologic) images

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Neoplastic Langerhans cells

Nodular infiltrates of Langerhans cells

Positive stains
Electron microscopy description
  • Intracytoplasmic Birbeck granules
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