Introduction The list of mediastinal masses in the paediatric age group includes a number of malignant and benign entities [1]. A very rare cause for a mediastinal mass is sclerosing mediastinitis. This condition results in fibrosis and sclerosis of the mediastinum, often leading to obstruction of intrathoracic organs. Only single paedi- atric cases of sclerosing mediastinitis have been reported in the medical literature to date [2]. Langerhans’ cell histiocytosis (LCH) is a reactive disorder characterised by infiltration of either single or multiple organ systems with proliferating cells of epi- dermal Langerhans’ cells [3]. According to one study, mediastinal mass as a manifestation of multisystem LCH is relatively rare (1 of 42 children), while pulmo- nary involvement without mediastinal mass is more frequent (8 of 42 children) [4]. We report the unusual course of a 2-year-old girl with LCH who initially presented with a solitary anterior mediastinal mass with SVC obstruction without calcifi- cation or pulmonary involvement. The radiological and histological findings are described. Case report A 2-year-old girl was admitted to our hospital with progressive signs of upper mediastinal vessel obstruction. Her growth and development were normal. The medical history disclosed an epi- sode of cough and dyspnoea 6 months previously. In another Andreas Trusen Matthias Beissert Helge Hebestreit Alexander Marx Kassa Darge Fibrosing mediastinitis with superior vena cava obstruction as the initial presentation of Langerhans’ cell histiocytosis in a young child Received: 28 February 2003 Accepted: 25 March 2003 Published online: 24 April 2003 Ó Springer-Verlag 2003 Abstract 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 re- ceived IV steroids for bronchial obstruction. On admission, clinical signs of SVC obstruction were evi- dent 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 histologi- cally interpreted as fibrosing medi- astinitis, and the final diagnosis of LCH was only made after identify- ing the skull lesion. Keywords Thorax Æ Mediastinum Æ Head Æ Skull Æ Langerhans’ cell histiocytosis Æ Fibrosing mediastinitis Æ Superior vena cava obstruction Pediatr Radiol (2003) 33: 485–488 DOI 10.1007/s00247-003-0929-2 CASE REPORT A. Trusen (&) Æ M. Beissert Æ K. Darge Department of Paediatric Radiology, Institute of Radiodiagnostics, University of Wu¨rzburg, Josef-Schneider-Straße 2, 97080 Wu¨rzburg, Germany E-mail: trusen@roentgen.uni-wuerzburg.de Tel.: +49-931-20134713 Fax: +49-931-20134857 H. Hebestreit University Children’s Hospital, University of Wu¨rzburg, Wu¨rzburg, Germany A. Marx Institute of Pathology, University of Wu¨rzburg, Wu¨rzburg, Germany