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Extraosseous Langerhans Cell Histiocytosis in Children¹

¹ From the Department of Diagnostic and Interventional Radiology (S.S., L.A.M., F.G.) and Department of Pathology (I.L.), Centre Hospitalier Universitaire Vaudois-CHUV, Rue du Bugnon, 1011 Lausanne, Switzerland; Department of Pediatric Radiology, Kinderspital, Aarau, Switzerland (G.E.); Fondation Lenval, Hôpital pour Enfants, Nice, France (A.G.); Department of Pediatric Radiology, Hôpital Cantonal, Geneva, Switzerland (S.H.); Department of Pediatric Radiology, Kinderspital, St. Gallen, Switzerland (P.W.); and Department of Pediatric Radiology, Inselspital, Bern, Switzerland (R.W.). Presented as an education exhibit at the 2006 RSNA Annual Meeting. Received May 10, 2007; revision requested July 12; revision received and accepted August 27. All authors have no financial relationships to disclose. Address correspondence to S.S. (e-mail: sabine.schmidt{at}chuv.ch).

Langerhans cell histiocytosis, a rare disease that occurs mainly in children, may produce a broad range of manifestations, from a single osseous lesion to multiple lesions involving more than one organ or system. The clinical course varies widely in relation to the patient’s age. Multisystem disease may demonstrate especially aggressive behavior in very young children, with the outcome depending largely on the stage of disease and the degree of related organ dysfunction at the time of diagnosis. Extraosseous manifestations are less commonly seen than osseous ones and may be more difficult to identify. To accurately detect extraosseous Langerhans cell histiocytosis at an early stage, radiologists must recognize the significance of individual clinical and laboratory findings as well as the relevance of imaging features for the differential diagnosis. The pattern and severity of pulmonary, thymic, hepatobiliary, splenic, gastrointestinal, neurologic, mucocutaneous, soft-tissue (head and neck), and salivary involvement in Langerhans cell histiocytosis generally are well depicted with conventional radiography, ultrasonography, computed tomography, and magnetic resonance imaging. However, the imaging features are not pathognomonic, and a biopsy usually is necessary to establish a definitive diagnosis.

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