title: Systemic juvenile xanthogranulomatosis imitating a malignant abdominal wall tumor with lung metastases.
abstract: Juvenile xanthogranulomatosis (JXG) is a rare benign condition, which usually presents with characteristic skin lesions and can be diagnosed clinically. However, systemic JXG may involve a wide range of extracutaneous sites and may pose a diagnostic dilemma for the clinician, radiologist, and pathologist. In particular it may simulate malignancy. Here, we report a case of systemic JXG within the abdominal wall musculature and lungs, which imitated a sarcoma with pulmonary metastases on computerized tomography. To the best of our knowledge, this is the first such reported case. In particular, systemic disease presenting without skin lesions is unusual.
authors.affiliation: Department of Paediatric Radiology, Bristol Royal Hospital for Children, Marlborough Street, Bristol BS2 8HW, UK. matchnj@doctors.org.uk
authors.forename: Nicola J
authors.initials: NJ
authors.lastname: Matcham
authors.forename: Savvas
authors.initials: S
authors.lastname: Andronikou
authors.forename: Keith
authors.initials: K
authors.lastname: Sibson
authors.forename: Pramila
authors.initials: P
authors.lastname: Ramani
authors.forename: David
authors.initials: D
authors.lastname: Grier
mesh_terms: D000008:Abdominal Neoplasms / Q000000981:diagnostic imaging*; D034861:Abdominal Wall; D002675:Child, Preschool; D005189:False Positive Reactions; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000000981:diagnostic imaging* / Q000556:secondary; D009362:Neoplasm Metastasis; D011859:Radiography; D012509:Sarcoma / Q000000981:diagnostic imaging; D015898:Tomography Scanners, X-Ray Computed; D014972:Xanthogranuloma, Juvenile / Q000000981:diagnostic imaging*