Extraosseous langerhans cell histiocytosis in children

Schmidt, Sabine; Eich, Georg; Geoffray, Anne; Hanquinet, Sylviane; Waibel, Peter; Wolf, Rainer; Letovanec, Igor; Alamo-Maestre, Leonore; Gudinchet, Francois (2008). Extraosseous langerhans cell histiocytosis in children. Radiographics, 28(3), 707-26; quiz 910-1. Easton, Pa.: Radiological Society of North America RSNA 10.1148/rg.283075108

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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.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Department of Radiology, Neuroradiology and Nuclear Medicine (DRNN) > Institute of Diagnostic, Interventional and Paediatric Radiology

UniBE Contributor:

Wolf, Rainer Walter

ISSN:

0271-5333

ISBN:

18480480

Publisher:

Radiological Society of North America RSNA

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 15:05

Last Modified:

04 May 2014 23:20

Publisher DOI:

10.1148/rg.283075108

PubMed ID:

18480480

Web of Science ID:

000255755200008

URI:

https://boris.unibe.ch/id/eprint/28164 (FactScience: 117771)

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