Langerhans cell histiocytosis
Progressive headache, ataxia and recent blindness.
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Multiple bilateral variable size supra tentorial intra-axial lesion in subcortical distribution showing low T1 signal, intermediated on T2 with homogenous enhancement associated with surrounding edema and white matter signal changes involving hypothalamic region, pons and dentate nuclei (high T2 and FLAIR) signal denoting neurodegenerative changes, no associated obstructive hydrocephalus, or skull bony lesions.
This patient went on to have a biopsy. Histopathology revealed infiltration of the lesions by a sheets of foamy histiocystes (xanthogranulomas) with plenty of multinucleated gaint cells including Touton giant cells and chronic inflammatory cells rich in mature lymphocytes and few plasma cells with traversing short bands of spindle fibroblast-like cells, findings are consistent with langerhans cell histiocytosis (CNS manifestations).
Langerhans cell histiocytosis is a systemic disorder involving the monocyte-macrophage system usually represent a systemic manifestation. CNS manifestation includes bony, intracranial hypothalamic-pituitary axis and pineal region, intracranial intra-axial lesions. The above case was initially diagnosed radiologically as CNS lymphoma or metastases.