Fact-sheet: Langerhans cell histiocytosis of the brain
Updated on 04/23/2019 at 9:45 AM
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Definition
Epidemiology:
- Incidence: 500 new cases per year in France
- Male/female ratio: 1.5
Systemic involvement:
- Primarily bone
- then to a lesser extent skin, ENT, liver, lung, lymph nodes, bone marrow/blood, spleen, nervous system
Clinical features
Several clinico-pathologic-radiologic forms of neuro-Langerhans cell histiocytosis:
- Tumoral
Increased intracranial pressure, seizures, focal deficit
- Degenerative
Cerebellar syndrome, pyramidal syndrome, impairment of higher cognitive functions
- Mixed
MRI
Forms:
- Tumoral
Most often a single lesion, involving the cerebral hemispheres, with homogeneous contrast enhancement
- Degenerative
often T1 hyperintensity of the dentate nuclei, occasionally the globus pallidus
cerebellar involvement
- Mixed
Management
Treatment:
- for tumoral forms: surgery when resectable, otherwise chemotherapy with VINBLASTINE
for degenerative forms:
- immunosuppressants, corticosteroids...
for mixed forms:
combined treatment