Document Detail

Multinodular and Vacuolating Neuronal Tumors of the Cerebrum: Ten cases of a distinctive seizure-associated lesion.
MedLine Citation:
PMID:  23324039     Owner:  NLM     Status:  Publisher    
We report 10 cases of a non-neurocytic, purely neuronal tumor affecting adults. Situated in the cerebral hemispheres, with 7 of 10 confined to the temporal lobes, most presented with seizures as their principal clinical manifestations. On MRI, the tumors generally appeared solid and non-contrast enhancing with minimal diffuse infiltration, edema, or mass effect. Six examples demonstrated internal nodularity. Microscopically, the tumor cells were largely distributed into discrete and coalescent nodules exhibiting varying degrees of matrix vacuolization, principally within the deep cortical ribbon and superficial subcortical white matter. Populating elements ranged from morphologically ambiguous to recognizably neuronal, with only two cases manifesting overt ganglion cell cytology. In all cases, tumor cells exhibited widespread nuclear immunolabeling for the HuC/HuD neuronal antigens, although expression of other neuronal markers, including synaptophysin, neurofilament, and chromogranin was variable to absent. Tumor cells also failed to express GFAP, p53, IDH1 R132H, or CD34, though CD34-labeling ramified neural elements were present in the adjoining cortex of 7 cases. Molecular analysis in a subset of cases failed to reveal DNA copy number abnormalities or BRAF V600E mutation. Follow-up data indicate that this unusual neuronal lesion behaves in benign, WHO grade I fashion and is amenable to surgical control.
Jason T Huse; Mark Edgar; John Halliday; Irina Mikolaenko; Ehud Lavi; Marc K Rosenblum
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-1-17
Journal Detail:
Title:  Brain pathology (Zurich, Switzerland)     Volume:  -     ISSN:  1750-3639     ISO Abbreviation:  Brain Pathol.     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-1-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9216781     Medline TA:  Brain Pathol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2013 The Authors; Brain Pathology © 2013 International Society of Neuropathology.
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY.
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