Child's Nervous System

, Volume 24, Issue 12, pp 1499–1503

Congenital subependymal giant cell astrocytoma: clinical considerations and expression of radial glial cell markers in giant cells

Authors

  • Ji Hoon Phi
    • Division of Pediatric NeurosurgerySeoul National University Children’s Hospital
  • Sung-Hye Park
    • Department of PathologySeoul National University Hospital
  • Jong Hee Chae
    • Department of PediatricsSeoul National University Children’s Hospital
  • Ki Ho Hong
    • Department of Laboratory MedicineSeoul National University Hospital
  • Sung Sup Park
    • Department of Laboratory MedicineSeoul National University Hospital
  • Ji Hyun Kang
    • Department of Obstetrics and GynecologySeoul National University Hospital
  • Jong Kwan Jun
    • Department of Obstetrics and GynecologySeoul National University Hospital
  • Byung-Kyu Cho
    • Division of Pediatric NeurosurgerySeoul National University Children’s Hospital
  • Kyu-Chang Wang
    • Division of Pediatric NeurosurgerySeoul National University Children’s Hospital
    • Division of Pediatric NeurosurgerySeoul National University Children’s Hospital
Case Report

DOI: 10.1007/s00381-008-0681-x

Cite this article as:
Phi, J.H., Park, S., Chae, J.H. et al. Childs Nerv Syst (2008) 24: 1499. doi:10.1007/s00381-008-0681-x

Abstract

Objects

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.

Case

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.

Keywords

Subependymal giant cell astrocytomaCongenitalTuberous sclerosis complexRadial glial cell

Copyright information

© Springer-Verlag 2008