Child's Nervous System

, Volume 24, Issue 12, pp 1499–1503 | Cite as

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

  • Ji Hoon Phi
  • Sung-Hye Park
  • Jong Hee Chae
  • Ki Ho Hong
  • Sung Sup Park
  • Ji Hyun Kang
  • Jong Kwan Jun
  • Byung-Kyu Cho
  • Kyu-Chang Wang
  • Seung-Ki Kim
Case Report

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 astrocytoma Congenital Tuberous sclerosis complex Radial glial cell 

Notes

Acknowledgment

This study was supported by a grant (no. 7-2005-1216; to Kim S. K.) from the Korean Ministry of Health and Welfare.

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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Ji Hoon Phi
    • 1
  • Sung-Hye Park
    • 2
  • Jong Hee Chae
    • 3
  • Ki Ho Hong
    • 4
  • Sung Sup Park
    • 4
  • Ji Hyun Kang
    • 5
  • Jong Kwan Jun
    • 5
  • Byung-Kyu Cho
    • 1
  • Kyu-Chang Wang
    • 1
  • Seung-Ki Kim
    • 1
  1. 1.Division of Pediatric NeurosurgerySeoul National University Children’s HospitalSeoulRepublic of Korea
  2. 2.Department of PathologySeoul National University HospitalSeoulRepublic of Korea
  3. 3.Department of PediatricsSeoul National University Children’s HospitalSeoulRepublic of Korea
  4. 4.Department of Laboratory MedicineSeoul National University HospitalSeoulRepublic of Korea
  5. 5.Department of Obstetrics and GynecologySeoul National University HospitalSeoulRepublic of Korea

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