Erdheim-Chester Disease of the Breast: A Case Report and Review of the Literature

:  Erdheim‐Chester disease (ECD) is a rare xanthomatous non‐Langerhans cell histiocytosis which involves the marrow space of the long bones. Extraosseous sites most commonly affected include the eyes, lungs, pituitary glands, and kidneys. We report the case of a 49‐year‐old woman who presented with...

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Bibliographic Details
Published in:The breast journal 2005-11, Vol.11 (6), p.462-467
Main Authors: Barnes, Penny J., Foyle, Annette, Haché, Kelly A. Dakin, Langley, Richard G. B., Burrell, Steven, Juskevicius, Ridas
Format: Article
Language:English
Subjects:
Adipose Tissue - pathology
Bone Diseases - pathology
breast
Breast Diseases - pathology
Deglutition Disorders - etiology
Diagnosis, Differential
Disease Progression
Dysarthria - etiology
Erdheim-Chester disease
Erdheim-Chester Disease - pathology
Female
histiocytosis
Humans
Middle Aged
Necrosis
Prognosis
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Summary::  Erdheim‐Chester disease (ECD) is a rare xanthomatous non‐Langerhans cell histiocytosis which involves the marrow space of the long bones. Extraosseous sites most commonly affected include the eyes, lungs, pituitary glands, and kidneys. We report the case of a 49‐year‐old woman who presented with palpable breast nodules, followed by progressive soft tissue and subcutaneous disease, and involvement of the long bones, dysarthria, and dysphagia. The histopathologic features and skeletal radiography findings are consistent with ECD. This case represents an unusual presentation, which led to delayed diagnosis, as ECD of the breast has been rarely reported. ECD should be considered in the differential diagnosis of histiocytoid breast lesions, including fat necrosis and histiocytoid invasive mammary carcinoma.
ISSN:1075-122X
1524-4741
DOI:10.1111/j.1075-122X.2005.00133.x