CNS tumor
Lymphoma and other hematopoietic lesions
Erdheim-Chester disease

Author: Nat Pernick, M.D. (see Authors page)

Revised: 7 September 2017, last major update January 2006

Copyright: (c) 2002-2017,, Inc.

PubMed Search: Erdheim-Chester disease of CNS

Cite this page: Pernick, N. Erdheim-Chester disease of CNS. website. Accessed May 21st, 2018.
Definition / general
  • Very rare ( < 100 cases reported), nonfamilial, neoplastic, xanthogranulomatous, non-Langerhans cell systemic histiocytosis first identified by William Chester in 1930
  • Usually associated with disease of long bones
  • Etiology not well understood
  • Mean age 57 years, range 25 - 76 years, no gender preference
  • Three year survival is 50 - 65%
  • Prognosis usually depends on extent of extraosseous disease
  • Rarely involves CNS
  • Usually hypothalamus and posterior pituitary (causing diabetes insipidus), retroorbital space
  • May affect dura
Radiology images

Images hosted on other servers:

Abnormal homogeneous enhancement

Homogeneously enhancing sellar mass

Case reports
  • 46 year old woman with brain stem infiltration by mixed Langerhans cell histiocytosis and Chester-Erdheim disease (Clin Exp Pathol 1999;47:71)
  • 55 year old woman with extracerebral subdural manifestation of Chester-Erdheim disease associated with a giant adenoma of pituitary (Clin Neuropathol 2003;22:246)
Microscopic (histologic) description
  • Diffuse infiltration with large, foamy histiocytes, lymphoid aggregates, fibrosis, rare Touton-like giant cells
Microscopic (histologic) images

Images hosted on other servers:

Histiocytic and
chronic inflammatory
lesion with
extensive fibrosis

Not CNS - pleural and interlobular septal infiltrate

Cytology description
  • Lymphohistiocytic elements and large multinucleated cells with abundant cytoplasm, vesicular nuclei and prominent nucleoli (Diagn Cytopathol 2004;31:420)
Positive stains
Negative stains
Electron microscopy description
  • Lipid droplets in cytoplasm but no Birbeck granules