Congenital subependymal giant cell astrocytoma: clinical considerations and expression of radial glial cell markers in giant cells.
OBJECTIVE
Congenital Subependymal giant cell astrocytoma (SEGA), diagnosed in fetal and neonatal period, is extremely rare. Previous studies have reported poor surgical outcomes of this small group of patients. We encountered a patient diagnosed as congenital SEGA and report the surgical outcome along with interesting immuno-phenotypes of giant tumor cells.


METHODS
Ventriculomegaly and a hypoechoic mass near the foramen of Monro were detected in a fetus on prenatal ultrasonography in the 35th week of gestation. Surgery was scheduled 2 months later to reduce the risk of operative complications. At postnatal 2 months, gross total resection of the tumor was achieved without complications. The patient had been followed up for 1 year without tumor recurrence. In double immunofluorescence, the prototype cells of SEGA expressed a variety of neural stem cell (nestin and Sox2) and radial glial cell markers (vimentin and brain lipid-binding protein), in addition to glutamate/aspartate transporter and glial fibrillary acidic protein.


CONCLUSIONS
Congenital SEGA can be successfully treated with judicious use of observation period and careful evaluation of general conditions. Pathological findings support the concept that SEGA may originate from aberrant radial glial cells in the developing brain.
Phi
Ji Hoon
JH
Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, 101 Daehangno, Jongno-gu, Seoul, 110-744, Republic of Korea.
Park
Sung-Hye
SH
Chae
Jong Hee
JH
Hong
Ki Ho
KH
Park
Sung Sup
SS
Kang
Ji Hyun
JH
Jun
Jong Kwan
JK
Cho
Byung-Kyu
BK
Wang
Kyu-Chang
KC
Kim
Seung-Ki
SK
D001254:Astrocytoma / Q000151:congenital / Q000175:diagnosis / Q000601:surgery*; D002352:Carrier Proteins / Q000096:biosynthesis; D002551:Cerebral Ventricle Neoplasms / Q000151:congenital / Q000175:diagnosis / Q000601:surgery*; D000071438:Fatty Acid-Binding Protein 7; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis / Q000378:metabolism / Q000601:surgery*; D005455:Fluorescent Antibody Technique / Q000379:methods; D006801:Humans; D007231:Infant, Newborn; D007381:Intermediate Filament Proteins / Q000096:biosynthesis; D008297:Male; D009419:Nerve Tissue Proteins / Q000096:biosynthesis; D064231:Nestin; D011247:Pregnancy; D011296:Prenatal Diagnosis; D055748:SOXB1 Transcription Factors / Q000096:biosynthesis; D025521:Tumor Suppressor Proteins / Q000096:biosynthesis; D016216:Ultrasonography, Prenatal; D014746:Vimentin / Q000096:biosynthesis
D002352:Carrier Proteins; C107608:FABP7 protein, human; D000071438:Fatty Acid-Binding Protein 7; D007381:Intermediate Filament Proteins; C577484:NES protein, human; D009419:Nerve Tissue Proteins; D064231:Nestin; C527782:SOX2 protein, human; D055748:SOXB1 Transcription Factors; D025521:Tumor Suppressor Proteins; D014746:Vimentin