GANGLIOGLIOMA (WHO I) GANGLIOGLIOMA (WHO I)
GANGLIOGLIOMA (WHO I) GANGLIOGLIOMA (WHO I)
GANGLIOGLIOMA (WHO I) GANGLIOGLIOMA (WHO I)
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LHERMITTE<br />
LHERMITTE-DUCLOS DUCLOS DZ (<strong>WHO</strong> I)<br />
� Dysplastic gangliocytoma of cerebellum<br />
� Peak onset in third to fourth decade<br />
� Internal granular layer dysplasia/hypertrophy<br />
� Expanded foliar cortex, diminished white matter<br />
� Obstructive hydrocephalus/ataxia<br />
� Cowden disease/PTEN mutations in nearly all<br />
adults, but some exceptions in children<br />
� PTEN functions in neuronal migration,<br />
development and soma size (knockout mice)<br />
LHERMITTE<br />
LHERMITTE-DUCLOS DUCLOS DZ<br />
LHERMITTE<br />
LHERMITTE-DUCLOS DUCLOS DZ LHERMITTE<br />
LHERMITTE-DUCLOS DUCLOS DZ<br />
LHERMITTE<br />
LHERMITTE-DUCLOS DUCLOS DZ<br />
CENTRAL NEUROCYTOMA (<strong>WHO</strong> II)<br />
� Older children/Young adults<br />
� Lat. v./3 rd v./F. of Monro/S. pellucidum<br />
� Obstructive hydrocephalus<br />
�� Globular/calcified<br />
� Usually benign, but may recur (MIB-1>2%)<br />
� DDx: oligodendroglioma, ependymoma<br />
� Genetics inconsistent, but no 1p/19q losses
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