title: Erdheim-Chester disease of the breast: a case report and review of the literature.
abstract: 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.
authors.affiliation: Division of Anatomical Pathology, Queen Elizabeth II Health Sciences Center, Halifax, Nova Scotia, Canada. Penny.Barnes@cdha.nshealth.ca
authors.forename: Penny J
authors.initials: PJ
authors.lastname: Barnes
authors.forename: Annette
authors.initials: A
authors.lastname: Foyle
authors.forename: Kelly A Dakin
authors.initials: KA
authors.lastname: Haché
authors.forename: Richard G B
authors.initials: RG
authors.lastname: Langley
authors.forename: Steven
authors.initials: S
authors.lastname: Burrell
authors.forename: Ridas
authors.initials: R
authors.lastname: Juskevicius
mesh_terms: D000273:Adipose Tissue / Q000473:pathology; D001847:Bone Diseases / Q000473:pathology; D001941:Breast Diseases / Q000473:pathology*; D003680:Deglutition Disorders / Q000209:etiology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004401:Dysarthria / Q000209:etiology; D031249:Erdheim-Chester Disease / Q000473:pathology*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009336:Necrosis; D011379:Prognosis