Document Detail

Tuber and subependymal giant cell astrocytoma associated with tuberous sclerosis: an immunohistochemical, ultrastructural, and immunoelectron and microscopic study.
MedLine Citation:
PMID:  8546029     Owner:  NLM     Status:  MEDLINE    
The cellular nature of the giant eosinophilic cells of tuber and of the cells comprising subependymal giant cell astrocytoma (SEGA) in tuberous sclerosis (TS) remains unclear. To assess the characteristics of these lesions, 13 tubers and 6 SEGA were immunohistochemically studied with glial and neuron-associated antigens. In addition to conventional ultrastructure, 6 tubers and 8 SEGA were subjected to immunoelectron microscopic study for glial fibrillary acidic protein (GFAP) and somatostatin. Eosinophilic giant cells of tubers were positive for vimentin (100%), GFAP (77%) and S-100 protein (92%); such cells were also found to a various extent to be reactive for neuron-associated antigens, including neurofilament (NF) proteins (38%) or class III beta-tubulin (77%). SEGA also showed variable immunoreactivity for GFAP (50%) or for S-100 protein (100%); NF epitopes, class III beta-tubulin, and calbindin 28-kD were expressed in 2 (33%), 5 (83%) and 4 (67%) cases, respectively. Cytoplasmic staining for somatostatin (50%), met-enkephalin (50%), 5-hydroxytryptamine (33%), beta-endorphin (33%) and neuropeptide Y (17%) was noted in SEGA, but not in tubers. Ultrastructurally, the giant cells of tubers and the cells of SEGA contained numerous intermediate filaments, frequent lysosomes and occasional rectangular or rhomboid membrane-bound crystalloids exhibiting lamellar periodicity and structural transition to lysosomes. Some SEGA cells showed features suggestive of neuronal differentiation, including stacks of rough endoplasmic reticulum, occasional microtubules and a few dense-core granules. Furthermore, in one case of tuber, a process of a single large cell was seen to be engaged in synapse formation. Intermediate filaments within a few cells of both lesions were decorated by gold particle-labeled GFAP antiserum. Within the tumor cells of SEGA, irregular, non-membrane-bound, electron-lucent areas often contained somatostatin-immunoreactive particles, whereas the latter could not be detected in tuber. The present study provides further evidence of divergent glioneuronal differentiation, both in the giant cells of tubers and the cells of SEGA. The findings of similar cells at different sites, including the subependymal zone, white matter ("heterotopias"), and cortex indirectly supports the idea that these lesions of TS result from a migration abnormality.
T Hirose; B W Scheithauer; M B Lopes; H A Gerber; H J Altermatt; M J Hukee; S R VandenBerg; J C Charlesworth
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Acta neuropathologica     Volume:  90     ISSN:  0001-6322     ISO Abbreviation:  Acta Neuropathol.     Publication Date:  1995  
Date Detail:
Created Date:  1996-02-13     Completed Date:  1996-02-13     Revised Date:  2007-11-09    
Medline Journal Info:
Nlm Unique ID:  0412041     Medline TA:  Acta Neuropathol     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  387-99     Citation Subset:  IM    
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA.
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MeSH Terms
Brain Neoplasms / pathology*,  ultrastructure
Cerebral Cortex / pathology
Child, Preschool
Glial Fibrillary Acidic Protein / metabolism
Glioma / pathology*,  ultrastructure
Microscopy, Immunoelectron
Somatostatin / metabolism
Tuberous Sclerosis / pathology*
Reg. No./Substance:
0/Glial Fibrillary Acidic Protein; 51110-01-1/Somatostatin

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