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

Child's Nervous System volume 34, pages 1511–1519 (2018)Cite this article

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.

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References

  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):36–44

    Article  PubMed  Google Scholar 

  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):158–174

    Article  PubMed  Google Scholar 

  3. Campen CJ, Porter BE (2011) Subependymal giant cell astrocytoma (SEGA) treatment update. Curr Treat Options Neurol 13(4):380–385

    Article  PubMed  PubMed Central  Google Scholar 

  4. Clarke MJ, Foy AB, Wetjen N, Raffel C (2006) Imaging characteristics and growth of subependymal giant cell astrocytomas. Neurosurg Focus 20(1):E5

    Article  PubMed  Google Scholar 

  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):232–243

    PubMed  Google Scholar 

  6. Curatolo P (1996) Neurological manifestations of tuberous sclerosis complex. Childs Nerv Syst 12(9):515–521

    Article  PubMed  CAS  Google Scholar 

  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):873–880

    Article  PubMed  Google Scholar 

  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):83–89

    Article  PubMed  Google Scholar 

  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):115–121

    Article  PubMed  Google Scholar 

  10. European Chromosome 16 Tuberous Sclerosis Consortium (1993) Identification and characterization of the tuberous sclerosis gene on chromosome 16. Cell 75(7):1305–1315

    Article  Google Scholar 

  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):125–132

    Article  PubMed  CAS  Google Scholar 

  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):45–48

    Article  PubMed  Google Scholar 

  13. Goh S, Butler W, Thiele EA (2004) Subependymal giant cell tumors in tuberous sclerosis complex. Neurology 63(8):1457–1461

    Article  PubMed  Google Scholar 

  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):21–28

    Article  PubMed  Google Scholar 

  15. Holanda FJ, Holanda GM (1980) Tuberous sclerosis—neurosurgical indications in intraventricular tumors. Neurosurg Rev 3(2):139–150

    Article  PubMed  CAS  Google Scholar 

  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):348–352

    Article  PubMed  Google Scholar 

  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):217–225

    Article  CAS  Google Scholar 

  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):1206–1213

    Article  PubMed  CAS  Google Scholar 

  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):1801–1811

    Article  PubMed  CAS  Google Scholar 

  20. Kumar R, Singh V (2004) Subependymal giant cell astrocytoma: a report of five cases. Neurosurg Rev 27(4):274–280

    Article  PubMed  Google Scholar 

  21. McLaurin RL, Towbin RB (1985–1986)) Tuberous sclerosis: diagnostic and surgical considerations. Pediatr Neurosci 12(1):43–48

    Article  PubMed  Google Scholar 

  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):370–375

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  23. Napolioni V, Moavero R, Curatolo P (2009) Recent advances in neurobiology of tuberous sclerosis complex. Brain Dev 31(2):104–113

    Article  PubMed  Google Scholar 

  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):31–34

    Article  PubMed  CAS  Google Scholar 

  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; 1993–2018

  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):243–254

    Article  PubMed  PubMed Central  Google Scholar 

  27. O’Callaghan FJ, Shiell AW, Osborne JP, Martyn CN (1998) Prevalence of tuberous sclerosis estimated by capture-recapture analysis. Lancet 351(9114):1490

    Article  PubMed  Google Scholar 

  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–S8

    Article  PubMed  CAS  Google Scholar 

  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):113–1121

    Article  Google Scholar 

  30. Roach ES, Gomez MR, Northrup H (1998) Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. J Child Neurol 13(12):624–628

    Article  PubMed  CAS  Google Scholar 

  31. Roszkowski M, Drabik K, Barszcz S, Jozwiak S (1995) Surgical treatment of intraventricular tumors associated with tuberous sclerosis. Childs Nerv Syst 11(6):335–339

    Article  PubMed  CAS  Google Scholar 

  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):439–444

    Article  PubMed  Google Scholar 

  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 26:28–31

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  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):219–224

    Article  PubMed  Google Scholar 

  35. Sinson G, Sutton LN, Yachnis AT, Duhaime AC, Schut L (1994) Subependymal giant cell astrocytomas in children. Pediatr Neurosurg 20(4):233–239

    Article  PubMed  CAS  Google Scholar 

  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):173–177

    Article  PubMed  CAS  Google Scholar 

  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):217–221

    Article  PubMed  CAS  Google Scholar 

  38. Van Slegtenhorst M, de Hoogt R, Hermans C et al (1997) Identification of the tuberous sclerosis gene TSC1 on chromosome 9q34. Science 277:805–808

    Article  PubMed  Google Scholar 

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Affiliations

  1. Department of Pediatric Neurosurgery, Fondation Ophtalmologique A. de Rothschild, Paris, France

    Martine Fohlen, Sarah Ferrand-Sorbets, Olivier Delalande & Georg Dorfmüller

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  1. Martine Fohlen
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  2. Sarah Ferrand-Sorbets
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  3. Olivier Delalande
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  4. Georg Dorfmüller
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Correspondence to Martine Fohlen.

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Fohlen, M., Ferrand-Sorbets, S., Delalande, O. et al. Surgery for subependymal giant cell astrocytomas in children with tuberous sclerosis complex. Childs Nerv Syst 34, 1511–1519 (2018). https://doi.org/10.1007/s00381-018-3826-6

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  • DOI: https://doi.org/10.1007/s00381-018-3826-6

Keywords

  • Hydrocephalus
  • Ventriculoperitoneal shunt
  • Pediatric
  • Brain tumor