Mediastinum
Hematological neoplasms / lesions
Langerhans cell histiocytosis

Author: Hanni Gulwani, M.D. (see Authors page)

Revised: 20 June 2018, last major update December 2012

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Langerhans cell histiocytosis mediastinum

Cite this page: Gulwani, H. Langerhans cell histiocytosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/mediastinumLCH.html. Accessed July 19th, 2018.
Definition / general
  • Rare neoplastic proliferation of Langerhans cells, a type of interdigitating dendritic cell which processes and presents antigens
Terminology
  • Also called Langerhans cell granulomatosis, histiocytosis X, eosinophilic granuloma, Letterer-Siwe disease, Hand-Schüller-Christian disease
Sites
  • 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

Images hosted on other servers:

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

Images hosted on other servers:

Neoplastic Langerhans cells

Nodular infiltrates of Langerhans cells

Positive stains
Electron microscopy description
  • Intracytoplasmic Birbeck granules