Document Detail

Fibrosing mediastinitis with superior vena cava obstruction as the initial presentation of Langerhans' cell histiocytosis in a young child.
MedLine Citation:
PMID:  12712269     Owner:  NLM     Status:  MEDLINE    
We present a 2-year-old girl with an unusual presentation of Langerhans' cell histiocytosis (LCH). Five months prior to admission to our hospital, she received IV steroids for bronchial obstruction. On admission, clinical signs of SVC obstruction were evident and a mediastinal mass was evident on the chest radiograph and MRI. Biopsy revealed fibrosing mediastinitis. Five months later, osteolysis was present on a skull radiograph. Surgical biopsy of the skull lesion revealed LCH. This case is unique because it demonstrates a rare initial manifestation of LCH that has not been previously reported. Furthermore, the primary, solitary mediastinal manifestation without calcifications was histologically interpreted as fibrosing mediastinitis, and the final diagnosis of LCH was only made after identifying the skull lesion.
Andreas Trusen; Matthias Beissert; Helge Hebestreit; Alexander Marx; Kassa Darge
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2003-04-24
Journal Detail:
Title:  Pediatric radiology     Volume:  33     ISSN:  0301-0449     ISO Abbreviation:  Pediatr Radiol     Publication Date:  2003 Jul 
Date Detail:
Created Date:  2003-06-23     Completed Date:  2003-11-18     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0365332     Medline TA:  Pediatr Radiol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  485-8     Citation Subset:  IM    
Department of Paediatric Radiology, Institute of Radiodiagnostics, University of Würzburg, Josef-Schneider-Strasse 2, 97080 Würzburg, Germany.
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MeSH Terms
Child, Preschool
Histiocytosis, Langerhans-Cell / complications*,  diagnosis
Magnetic Resonance Imaging
Mediastinitis / diagnosis,  etiology*,  radiography
Skull / radiography
Superior Vena Cava Syndrome / etiology*,  radiography

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