Lymph nodes & spleen, nonlymphoma
Lymph node & spleen-nonlymphoid neoplasms
Erdheim-Chester disease
Author: Patricia Tsang, M.D., M.B.A.
Last author update: 1 May 2014
Last staff update: 6 October 2020
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed Search: Erdheim-Chester disease[TI] free full text[sb]
See also: Bone chapter
Table of Contents
Definition / general | Clinical features | Radiology description | Radiology images | Case reports | Microscopic (histologic) description | Microscopic (histologic) images | Cytology images | Positive stains | Negative stains | Differential diagnosis | Additional referencesCite this page: Tsang P. Erdheim-Chester disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphnodeserdheimchester.html. 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
-
Lymph node:
- 49 year old woman with lymphadenpathy, tender skin papules and a pathologic tibial fracture (Case of the Week #299)
- 50 year old man with Erdheim-Chester disease (J Clin Pathol 2004;57:1225)
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
CD163+
Cytology images
Images hosted on other servers:
Histiocytes from touch preparation
Positive stains
Differential diagnosis
Additional references
