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Child's Nervous System

, Volume 34, Issue 8, pp 1511–1519 | Cite as

Surgery for subependymal giant cell astrocytomas in children with tuberous sclerosis complex

  • Martine Fohlen
  • Sarah Ferrand-Sorbets
  • Olivier Delalande
  • Georg Dorfmüller
Original Paper

Abstract

Objective

Subependymal giant cell astrocytomas (SEGAs) are low-grade intraventricular glial tumors that develop in 10–15% of patients with tuberous sclerosis complex; they often cause hydrocephalus and are potentially accessible to a surgical treatment. Our aim is to evaluate morbidity and results after surgery in symptomatic and asymptomatic patients.

Method

We present a retrospective series of 18 pediatric patients operated on for SEGA between 2006 and 2016 at our institution. We reviewed surgical indications, preoperative clinical and radiologic data, surgical management, and clinical and radiological follow-up.

Results

Mean age at surgery was 10.7 years. The surgical decision was based on clinical signs of raised intracranial pressure due to hydrocephalus in 8 and on radiological findings without any clinical signs in the other 10 patients (increased in SEGA volume with or without ventricular enlargement). Surgical treatment consisted in a frontal trans-ventricular microsurgical approach in 17 patients and an endoscopic approach in 1. External ventricular drainage was placed in all the patients but 1. Ventriculoperitoneal shunting (VPS) became necessary in 6 patients, all of them presenting with a preoperative active hydrocephalus. Morbidity appeared very low with meningitis occurring in 1 patient. Resection was complete in 15 children with no recurrence during a mean follow-up of 5.25 years and incomplete in 3 requiring a second surgery.

Conclusion

Surgery of SEGA represents a very effective treatment with low morbidity and no mortality in the present series. In patients operated before the onset of clinical signs of hydrocephalus, internal VPS could be avoided whereas in others, an additional shunt surgery became necessary. This gives arguments in favor of a regular MRI surveillance in tuberous sclerosis complex patients with SEGA in order to best propose resective surgery once a growth of tumor and/or ventricular size have been confirmed but before raised intracranial pressure occurs.

Keywords

Hydrocephalus Ventriculoperitoneal shunt Pediatric Brain tumor 

Notes

Compliance with ethical standards

Conflict of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

References

  1. 1.
    Amin S, Carter M, Edwards RJ, Pople I, Aquilina K, Merrifield J, Osborne JP, O'Callaghan FJK (2013) The outcome of surgical management of subependymal giant cell astrocytoma in tuberous sclerosis complex. Eur J Paediatr Neurol 17(1):36–44CrossRefPubMedGoogle Scholar
  2. 2.
    Blümcke I, Thom M, Aronica E, Armstrong DD, Vinters HV, Palmini A, Jacques TS, Avanzini G, Barkovich AJ, Battaglia G, Becker A, Cepeda C, Cendes F, Colombo N, Crino P, Cross JH, Delalande O, Dubeau F, Duncan J, Guerrini R, Kahane P, Mathern G, Najm I, Özkara Ç, Raybaud C, Represa A, Roper SN, Salamon N, Schulze-Bonhage A, Tassi L, Vezzani A, Spreafico R (2011) The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission. Epilepsia 52(1):158–174CrossRefPubMedGoogle Scholar
  3. 3.
    Campen CJ, Porter BE (2011) Subependymal giant cell astrocytoma (SEGA) treatment update. Curr Treat Options Neurol 13(4):380–385CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Clarke MJ, Foy AB, Wetjen N, Raffel C (2006) Imaging characteristics and growth of subependymal giant cell astrocytomas. Neurosurg Focus 20(1):E5CrossRefPubMedGoogle Scholar
  5. 5.
    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):232–243PubMedGoogle Scholar
  6. 6.
    Curatolo P (1996) Neurological manifestations of tuberous sclerosis complex. Childs Nerv Syst 12(9):515–521CrossRefPubMedGoogle Scholar
  7. 7.
    Curatolo P, Napolioni V, Moavero R (2010) Autism spectrum disorders in tuberous sclerosis: pathogenetic pathways and implications for treatment. J Child Neurol 25(7):873–880CrossRefPubMedGoogle Scholar
  8. 8.
    De Ribaupierre S, Dorfmüller G, Bulteau C et al (2007) Subependymal giant-cell astrocytomas in pediatric tuberous sclerosis disease: when should we operate? Neurosurgery 60(1):83–89CrossRefPubMedGoogle Scholar
  9. 9.
    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(3):115–121CrossRefPubMedGoogle Scholar
  10. 10.
    European Chromosome 16 Tuberous Sclerosis Consortium (1993) Identification and characterization of the tuberous sclerosis gene on chromosome 16. Cell 75(7):1305–1315CrossRefGoogle Scholar
  11. 11.
    Franz DN, Belousova E, Sparagana S, Bebin EM, Frost M, Kuperman R, Witt O, Kohrman MH, Flamini JR, Wu JY, Curatolo P, de Vries PJ, Whittemore VH, Thiele EA, Ford JP, Shah G, Cauwel H, Lebwohl D, Sahmoud T, Jozwiak S (2013) Efficacy and safety of everolimus for subependymal giant cell astrocytomas associated with tuberous sclerosis complex (EXIST-1): a multicentre, randomised, placebo-controlled phase 3 trial. Lancet 381(9861):125–132CrossRefPubMedGoogle Scholar
  12. 12.
    Frèrebeau P, Benezech J, Segnarbieux F, Harbi H, Desy A, Marty-Double C (1985) Intraventricular tumors in tuberous sclerosis. Childs Nerv Syst 1(1):45–48CrossRefPubMedGoogle Scholar
  13. 13.
    Goh S, Butler W, Thiele EA (2004) Subependymal giant cell tumors in tuberous sclerosis complex. Neurology 63(8):1457–1461CrossRefPubMedGoogle Scholar
  14. 14.
    Harter DH, Bassani L, Rodgers SD, Roth J, Devinsky O, Carlson C, Wisoff JH, Weiner HL (2014) A management strategy for intraventricular subependymal giant cell astrocytomas in tuberous sclerosis complex. J Neurosurg Pediatr 13(1):21–28CrossRefPubMedGoogle Scholar
  15. 15.
    Holanda FJ, Holanda GM (1980) Tuberous sclerosis—neurosurgical indications in intraventricular tumors. Neurosurg Rev 3(2):139–150CrossRefPubMedGoogle Scholar
  16. 16.
    Jóźwiak S, Nabbout R, Curatolo P, participants of the TSC Consensus Meeting for SEGA and Epilepsy Management (2013) Management of subependymal giant cell astrocytoma (SEGA) associated with tuberous sclerosis complex (TSC): clinical recommendations. Eur J Paediatr Neurol 17(4):348–352CrossRefPubMedGoogle Scholar
  17. 17.
    Kim SK, Wang KC, Cho BK, Jung HW, Jin Lee Y, Seob Chung Y, Lee JY, Park SH, Kim YM, Choe G, Chi JG (2001) Biological behavior and tumorigenesis of subependymal giant cell astrocytomas. J Neuro-Oncol 52(3):217–225CrossRefGoogle Scholar
  18. 18.
    Koh S, Jayakar P, Dunoyer C, Whiting SE, Resnick TJ, Alvarez LA, Morrison G, Ragheb J, Prats A, Dean P, Antel SB, Gilman J, Duchowny MS (2000) Epilepsy surgery in children with tuberous sclerosis complex: presurgical evaluation and outcome. Epilepsia 41(9):1206–1213CrossRefPubMedGoogle Scholar
  19. 19.
    Krueger DA, Care MM, Holland K, Agricola K, Tudor C, Mangeshkar P, Wilson KA, Byars A, Sahmoud T, Franz DN (2010) Everolimus for subependymal giant-cell astrocytomas in tuberous sclerosis. N Engl J Med 363(19):1801–1811CrossRefPubMedGoogle Scholar
  20. 20.
    Kumar R, Singh V (2004) Subependymal giant cell astrocytoma: a report of five cases. Neurosurg Rev 27(4):274–280CrossRefPubMedGoogle Scholar
  21. 21.
    McLaurin RL, Towbin RB (1985–1986)) Tuberous sclerosis: diagnostic and surgical considerations. Pediatr Neurosci 12(1):43–48CrossRefPubMedGoogle Scholar
  22. 22.
    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):370–375CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Napolioni V, Moavero R, Curatolo P (2009) Recent advances in neurobiology of tuberous sclerosis complex. Brain Dev 31(2):104–113CrossRefPubMedGoogle Scholar
  24. 24.
    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):31–34CrossRefPubMedGoogle Scholar
  25. 25.
    Northrup H, Koenig MK, Pearson DA, Au KS (1999) In: Adam MP et al (ed) Tuberous sclerosis complex GeneReviews Seattle (WA): University of Washington, Seattle; 1993–2018Google Scholar
  26. 26.
    Northrup H, Krueger DA, International Tuberous Sclerosis Complex Consensus Group (2013) Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol 49(4):243–254CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    O’Callaghan FJ, Shiell AW, Osborne JP, Martyn CN (1998) Prevalence of tuberous sclerosis estimated by capture-recapture analysis. Lancet 351(9114):1490CrossRefPubMedGoogle Scholar
  28. 28.
    Palmini A, Najm I, Avanzini G, Babb T, Guerrini R, Foldvary-Schaefer N, Jackson G, Luders HO, Prayson R, Spreafico R, Vinters HV (2004) Terminology and classification of the cortical dysplasias. Neurology 62(6 Suppl 3):S2–S8CrossRefPubMedGoogle Scholar
  29. 29.
    Ren H, Chen X, Sun G et al (2013) Resection of subependymal giant cell astrocytoma guided by intraoperative magnetic resonance imaging and neuronavigation. Childs Nerv Syst 29(7):113–1121CrossRefGoogle Scholar
  30. 30.
    Roach ES, Gomez MR, Northrup H (1998) Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. J Child Neurol 13(12):624–628CrossRefPubMedGoogle Scholar
  31. 31.
    Roszkowski M, Drabik K, Barszcz S, Jozwiak S (1995) Surgical treatment of intraventricular tumors associated with tuberous sclerosis. Childs Nerv Syst 11(6):335–339CrossRefPubMedGoogle Scholar
  32. 32.
    Roth J, Roach ES, Bartels U, Jóźwiak S, Koenig MK, Weiner HL, Franz DN, Wang HZ (2013) Subependymal giant cell astrocytoma: diagnosis, screening, and treatment. Recommendations from the International Tuberous Sclerosis Complex Consensus Conference 2012. Pediatr Neurol 49(6):439–444CrossRefPubMedGoogle Scholar
  33. 33.
    Sampson J, Scahill S, Stephenson J, Mann L, Connor J (1989) Genetic aspects of tuberous sclerosis in the west of Scotland. J Med Genet 26:28–31CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Sharma MC, Ralte AM, Gaekwad S, Santosh V, Shankar SK, Sarkar C (2004) Subependymal giant cell astrocytoma—a clinicopathological study of 23 cases with special emphasis on histogenesis. Pathol Oncol Res 10(4):219–224CrossRefPubMedGoogle Scholar
  35. 35.
    Sinson G, Sutton LN, Yachnis AT, Duhaime AC, Schut L (1994) Subependymal giant cell astrocytomas in children. Pediatr Neurosurg 20(4):233–239CrossRefPubMedGoogle Scholar
  36. 36.
    Torres OA, Roach ES, Delgado MR, Sparagana SP, Sheffield E, Swift D, Bruce D (1998) Early diagnosis of subependymal giant cell astrocytoma in patients with tuberous sclerosis. J Child Neurol 13(4):173–177CrossRefPubMedGoogle Scholar
  37. 37.
    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(3):217–221CrossRefPubMedGoogle Scholar
  38. 38.
    Van Slegtenhorst M, de Hoogt R, Hermans C et al (1997) Identification of the tuberous sclerosis gene TSC1 on chromosome 9q34. Science 277:805–808CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Martine Fohlen
    • 1
  • Sarah Ferrand-Sorbets
    • 1
  • Olivier Delalande
    • 1
  • Georg Dorfmüller
    • 1
  1. 1.Department of Pediatric NeurosurgeryFondation Ophtalmologique A. de RothschildParisFrance

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