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Target Concepts:
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Query: UMLS:C0019621 (
Langerhans cell histiocytosis
)
3,250
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
A case of
Langerhans' cell histiocytosis
confined to the mediastinum and presenting with de novo superior cava syndrome is reported. The causes of
superior vena cava syndrome
in childhood are discussed as is the importance of obtaining pathologic diagnosis prior to initiating therapy.
...
PMID:Langerhans' cell histiocytosis presenting with the superior vena cava syndrome: a case report. 851 29
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.
...
PMID:Fibrosing mediastinitis with superior vena cava obstruction as the initial presentation of Langerhans' cell histiocytosis in a young child. 1271 69
