Lymph nodes & spleen, nonlymphoma

Lymph node & spleen-nonlymphoid neoplasms

Erdheim-Chester disease

Last author update: 1 May 2014
Last staff update: 6 October 2020

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PubMed Search: Erdheim-Chester disease[TI] free full text[sb]

See also: Bone chapter

Patricia Tsang, M.D., M.B.A.
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Cite this page: Tsang P. Erdheim-Chester disease. website. Accessed June 5th, 2023.
Definition / general
  • Rare non-Langerhans cell histiocytosis of bone of unknown etiology, with 50% also involving hypothalamic pituitary axis, lung, heart, retroperitoneum, skin, liver, kidneys, spleen, orbit
  • Nodal involvement is very rare, despite multisystem disease
Clinical features
  • Most common symptom is bone pain, usually involving lower extremities
  • Broad extraskeletal manifestations, including fever, weight loss, weakness, exophthalmos, diabetes insipidus, dysuria, abdominal pain, obstructive renal impairment, ataxia and paresis
  • Interstitial lung disease in 35% of patients with histiocytic cells and fibrosis seen in a predominantly perilymphangitic and subpleural pattern
  • Prognosis depends on extent and distribution of extraskleletal manifestations
  • Most common causes of death are respiratory distress, extensive pulmonary fibrosis and cardiac failure
Radiology description
  • Pathognomonic radiography showing bilateral, symmetrical osteosclerosis of metaphyseal and diaphyseal regions and sparing of the epiphyses
Radiology images

Case #299

Pathologic fracture of left tibia

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
    • Large foamy histiocytes containing lipid, lymphocytic aggregates, fibrosis
    • Rare Touton-like giant cells
    Microscopic (histologic) images

    Case #299

    Neck lymph node biopsy

    Skin lesion biopsy


    Cytology images

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    Histiocytes from touch preparation

    Positive stains
    Negative stains
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