Skip to main content

Advertisement

SpringerLink
Log in

Subependymal giant cell astrocytoma: a report of five cases

  • Neurooncology
  • Published:
Neurosurgical Review Aims and scope Submit manuscript

Abstract

Five cases of intraventricular subependymal giant cell astrocytomas (SEGCA) were retrospectively reviewed. Records and detailed work-up of all five cases were well maintained in view of rare occurrence of these tumors. The five patients were males aged 10, 12 (2 cases), 14 and 18 years. The 18-year-old aged male had no stigmata of tuberous sclerosis (TSC) on examination, while the rest presented with features of TSC, intraventricular tumors and hydrocephalus. None of the five cases had renal tumors and did not reveal cardiac tumors. Skin manifestations like shagreen patches were present in two, facial angiofibromas in three, periungal fibromas in two, hypopigmented macules in two and ash-leaf spot in two patients. Fundoscopic examination revealed retinal astrocytomas in two cases only. Two of the five children did not have mental compromise. A child who started throwing seizures early (at 5 months of age) had severe mental retardation. Although SEGCA is a less vascular tumor, one tumor bled profusely intraoperatively; it was excised rapidly via the transcortical route to salvage the life of the child, though he was planned for the transcallosal route like the other cases. The tumor of another child was also very vascular. Four children survived surgery and required no shunt CSF diversion, while one died of severe ventriculitis and septicemia 3 weeks after surgery.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2 Fig. 3 Fig. 4

Similar content being viewed by others

References

  1. Hahn JS, Bejar R, Gladson CL (1991) Neonatal subependymal giant cell astrocytoma associated with tuberous sclerosis: MRI, CT and ultrasound correlation. Neurology 41:124–128

    CAS  PubMed  Google Scholar 

  2. Nabbout R, Santos M, Rolland Y, Delalande O, Dulac O, Chiron C (1999) Early diagnosis of subependymal giant cell astrocytoma in children with tuberous sclerosis. J Neurol Neurosurg Psychiatry 66(3):370–375

    CAS  PubMed  Google Scholar 

  3. Sinson G, Sutton LN, Yachnis AT, Duhaime AC, Shut L (1994) Subependymal giant cell astrocytomas in children. Pediatr Neurosurg 20:233–239

    CAS  PubMed  Google Scholar 

  4. Watanabe Y, Oki S, Migita K, Isobe N, Okazaki T, Nabika S (2003) A case of subependymal giant cell astrocytoma not associated with tuberous sclerosis. No Shinkei Geka 31(5):543–548

    PubMed  Google Scholar 

  5. Altman NR, Purser RK, Post MJD (1988) Tuberous sclerosis: Characteristics on CT and MR imaging. Radiology 167:527–532

    CAS  PubMed  Google Scholar 

  6. Curatolo P (1996) Neurological manifestations of tuberous sclerosis complex. Childs Nerv Syst 12:515–521

    CAS  PubMed  Google Scholar 

  7. Griffiths PD, Martland TR (1997) Tuberous sclerosis complex: the role of neuroradiology. Neuropediatrics 28:244–252

    CAS  PubMed  Google Scholar 

  8. Cuccia V, Zuccaro G, Sosa F, Monges J, Lubienieky F, Taratuto AL (2003) Subependymal giant cell astrocytoma in children with tuberous sclerosis. Childs Nerv Syst 19(4):232–243

    PubMed  Google Scholar 

  9. Roach ES, Smith M, Hutterlocher P et al (1992) Diagnostic criteria—tuberous sclerosis. J Child Neurol 7:221–224

    CAS  PubMed  Google Scholar 

  10. Gomez M (1995) History of the tuberous sclerosis complex. Brain Dev 17:55–57

    Article  PubMed  Google Scholar 

  11. Earnest L, Byrne P, Califano J (2003) Massive facial angioma in a patient with tuberous sclerosis. Otolaryngol Head Neck Surg 128(1):151–153

    Article  PubMed  Google Scholar 

  12. Aste N, Pau M, Biggio P (2003) Tuberous sclerosis with unusual giant ungual fibromas. J Eur Acad Dermatol Venereol 17(1):100–101

    Article  CAS  PubMed  Google Scholar 

  13. Fryer AE, Osborne JP, Schutt W (1987) Forehead plaque: a presenting skin sign in tuberous sclerosis. Arch Dis Child 62:292–293

    CAS  PubMed  Google Scholar 

  14. Hass-Teller C, Rohrwacher F, Wiedemann P (1998) Bilateral retinal tumours in a child. Tuberous sclerosis with retinal astrocytomas. Ophthalmologe 95(5):348–349

    Article  CAS  PubMed  Google Scholar 

  15. Jansen FE, Vles H, Van Nieuwenhuizen O (2002) Epileptic blindness in tuberous sclerosis complex. Dev Med Child Neurol 44(11):792

    Article  CAS  PubMed  Google Scholar 

  16. Simmons JL, Hussain SA, Riley P, Wallace DM (2003) Management of renal angiomyolipoma in patients with tuberous sclerosis complex. Oncol Rep 10(1):237–241

    CAS  PubMed  Google Scholar 

  17. Smith HC, Watson GH, Palel RG et al (1989) Cardiac rhabdomyomata in tuberous sclerosis: Their course and diagnostic value. Arch Dis Child 64:196

    CAS  PubMed  Google Scholar 

  18. Webb DW, Thomas RD, Osborne JP (1993) Cardiac rhabdomyomas and their association with tuberous sclerosis. Arch Dis Child 68:367–370

    CAS  PubMed  Google Scholar 

  19. Harding CO, Pagon RA (1990) Incidence of tuberous sclerosis in patients with cardiac rhabdomyoma. Am J Med Genet 37:443–446

    CAS  PubMed  Google Scholar 

  20. Noguchi N, Kanno H, Sakata K, Tokoro K, Yamamoto I (1997) Tuberous sclerosis associated with subependymal giant cell astrocytoma and renal angiomyolipoma: a case report. No Shinkei Geka 25(11):1017–1019

    CAS  PubMed  Google Scholar 

  21. Nishio S, Morioka T, Suzuki S, Kira R, Mihara F, Fukui M (2001) Subependymal giant cell astrocytoma: clinical and neuroimaging features of four cases. J Clin Neurosci 8(1):31–34

    Article  CAS  PubMed  Google Scholar 

  22. Frerebeau PH, Benezech J, Segnarbieux F et al (1985) Intraventricular tumors in tuberous sclerosis. Childs Nerv Syst 1:45–48

    CAS  PubMed  Google Scholar 

  23. Yamamoto K, Yamada K, Nakahara T, Ishihara A, Takaki S, Kochi M, Ushio Y (2002) Rapid regrowth of solitary subependymal giant cell asrtocytoma, case report. Neurol Med Chir (Tokyo) 42(5):224–227

    Google Scholar 

  24. Kim SK, Wang KC, Cho BK, Jung HW et al (2001) Biological behavior and tumourigenesis of subependymal giant cell astrocytomas. J Neurooncol 52(3):217–225

    Article  CAS  PubMed  Google Scholar 

  25. Hosoya M, Naito H, Nihei K (1999) Neurological prognosis correlated with variations over time in the number of subependymal nodules in tuberous sclerosis. Brain Dev 21(8):544–547

    Article  CAS  PubMed  Google Scholar 

  26. Mehmet T, Nejat A, Tuncalp O et al (1996) Subependymal giant cell astrocytoma associated with tuberous sclerosis: diagnostic and surgical characteristics of five cases with unusual features. Clin Neurol Neurosurg 98:217–221

    Article  PubMed  Google Scholar 

  27. Roszkowaski M, Drabik K, Barszcz S et al (1995) Surgical treatment of intraventricular tumors associated with tuberous sclerosis. Childs Nerv Syst 11:335–339

    PubMed  Google Scholar 

  28. Beems T, Grotenhuis JA (2001) Subependymal giant-cell astrocytoma in tuberous sclerosis: endoscopic images and the implications for therapy. Minim Invasive Neurosurg 44(1):58–60

    Article  CAS  PubMed  Google Scholar 

  29. Shepherd CW, Scheithauer BW, Gomez MR et al (1991) Subependymal giant cell astrocytoma: a clinical, pathological and flow cytometric study. Neurosurgery 28:864–868

    PubMed  Google Scholar 

  30. Di Rocco C, Iannelli A, Marchese E (1995) On the treatment of subependymal giant cell astrocytomas and associated hydrocephalus in tuberous sclerosis. Pediatr Neurosurg 23:115–121

    PubMed  Google Scholar 

  31. Turgut M, Akalan N, Ozgen T, Ruacan S, Erbengi A (1996) Subependymal giant cell astrocytoma associated with tuberous sclerosis: diagnostic and surgical characteristics of five cases with unusual features. Clin Neurol Neurosurg 98:217–221

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

The authors are extremely grateful to Mr A. P. Dhar Dwivedi for the preparation of this manuscript.

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow-226014, UP, India

    Raj Kumar

  2. Department of Anesthesiology, King George’s Medical University, Lucknow, India

    Vinita Singh

Authors
  1. Raj Kumar
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Vinita Singh
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Raj Kumar.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kumar, R., Singh, V. Subependymal giant cell astrocytoma: a report of five cases. Neurosurg Rev 27, 274–280 (2004). https://doi.org/10.1007/s10143-004-0339-4

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10143-004-0339-4

Keywords

Search

Navigation