A 5 Y/O Female with Langerhans cell histiocytosis
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Result of abnormal prolifration of langerhans histiocytes
Most frequently in childrens
Has extremely variable presentation:
• Letterer-Siwe (acute disseminated form): 10%
• Hand-Schuller-Christian (chronic disseminated form):20%
• Eosinophilic granuloma (isolated bone or lung involvement): 70%
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Best diagnostic clue:
o Skull :sharply marginated lytic skull defect with beveled margins
o Mastoid :geographic destruction, soft tissue mass
o Brain :thick enhancing infundibulum
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Comment
lateral skull x.ray showed:-
- large radiolucent macrolobulated area at the tempro-occipital region,involved sphenoid bone and the clvarial tablets.
- widening the diploic area in clvarium of skull,with lucent part, and sclerotic part.
-all these consistant with bengin condition of bony lesion likely bone marrow tumer.
- sclerotic sphenoid bone.with thichening cortical bone no erosion.
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