PMID- 18459885
OWN - NLM
STAT- MEDLINE
DCOM- 20090210
LR  - 20220331
IS  - 0022-3085 (Print)
IS  - 0022-3085 (Linking)
VI  - 107
IP  - 2 Suppl
DP  - 2007 Aug
TI  - Anaplastic ganglioglioma arising from a Lhermitte-Duclos-like lesion. Case 
      report.
PG  - 137-42
LID - 10.3171/PED-07/08/137 [doi]
AB  - The authors report the case of a 7-year-old boy with a history of developmental 
      delay who presented with aggressive behavior. A magnetic resonance (MR) image 
      showed a mass lesion originating from the cerebellar vermis with an atypical 
      folial pattern and contrast enhancement. Histologically, the subtotally resected 
      specimen consisted mostly of neuropil with nodular foci of ganglion cells. 
      Lhermitte-Duclos disease (LDD) was diagnosed in the patient. A retrospective 
      review of the tissue sections showed a nidus of associated astrocytic 
      proliferation, suggesting a diagnosis of ganglioglioma. Five years later, the 
      patient experienced an altered mental state and a facial droop. An MR image 
      revealed a cerebellar mass with cystic areas and an enhancing nodule. The 
      resected tissue specimen consisted primarily of a mixed proliferation of glial 
      and ganglion cells consistent with a ganglioglioma. Two years later, a third 
      craniectomy was performed in the patient for worsening headache and ataxia. 
      Histologically, the tumor showed progressive anaplasia and was most accurately 
      classified as an anaplastic ganglioglioma. Immunohistochemically, most of the 
      tumor cells were immunoreactive for anti-phospho-mammalian target of rapamycin 
      (mTOR) and phospho-S6 ribosomal protein antibodies. In contrast, the 
      subpopulation of neoplastic ganglion cells in the tissue, particularly from the 
      first surgery, did not express phosphatase and tensin homolog deleted from 
      chromosome 10 (PTEN). This immunohistochemical pattern suggests that the large 
      dysplastic ganglion cells (the gangliocytomatous component) forming the greater 
      part of the lesion were associated with activation of the phosphatidylinositol 
      3-kinase-PTEN/Akt/mTOR signaling pathway, a feature previously reported in LDD. 
      This case represents the first report of an anaplastic ganglioglioma arising in 
      an LDD-like lesion.
FAU - Takei, Hidehiro
AU  - Takei H
AD  - Department of Pathology, Texas Children's Cancer Center, Baylor College of 
      Medicine, USA. takei327@aol.com
FAU - Dauser, Robert
AU  - Dauser R
FAU - Su, Jack
AU  - Su J
FAU - Chintagumpala, Murali
AU  - Chintagumpala M
FAU - Bhattacharjee, Meenakshi B
AU  - Bhattacharjee MB
FAU - Jones, Jeremy
AU  - Jones J
FAU - Adesina, Adekunle M
AU  - Adesina AM
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - J Neurosurg
JT  - Journal of neurosurgery
JID - 0253357
SB  - IM
MH  - Anaplasia/pathology/psychology/surgery
MH  - Cerebellar Neoplasms/*pathology/psychology/surgery
MH  - Child
MH  - Ganglioglioma/*pathology/psychology/surgery
MH  - Hamartoma Syndrome, Multiple/*pathology
MH  - Humans
MH  - Male
EDAT- 2008/05/10 09:00
MHDA- 2009/02/12 09:00
CRDT- 2008/05/10 09:00
PHST- 2008/05/10 09:00 [pubmed]
PHST- 2009/02/12 09:00 [medline]
PHST- 2008/05/10 09:00 [entrez]
AID - 10.3171/PED-07/08/137 [doi]
PST - ppublish
SO  - J Neurosurg. 2007 Aug;107(2 Suppl):137-42. doi: 10.3171/PED-07/08/137.