Child's Nervous System

, Volume 29, Issue 2, pp 335–339 | Cite as

Subependymal giant cell astrocytoma associated with tuberous sclerosis presenting with intratumoral bleeding. Case report and review of literature

  • Hugo Sterman
  • Andre Beer Furlan
  • Hamilton Matushita
  • Manoel Jacobsen Teixeira
Case Report


The authors describe a rare case of subependymal giant cell astrocytoma (SEGA) associated with tuberous sclerosis complex that presented with intratumoral bleeding with extension to the ventricles. The literature regarding intracranial hemorrhage of SEGA is reviewed and only five cases have been reported in the literature. We have not identified a histological feature associated with bleeding.


Subependymal giant cell astrocytoma Tuberous sclerosis Intratumoral hemorrhage Intraventricular hemorrhage 



The authors report that they did not have any conflict of interest concerning the material or methods used in this study or the findings specified in this paper.


  1. 1.
    Al-Jarallah A, Al-Rifai MT, Riela AR, Roach ES (2000) Nontraumatic brain hemorrhage in children: etiology and presentation. J Child Neurol 15:284–289PubMedCrossRefGoogle Scholar
  2. 2.
    Arbiser JL, Brat D, Hunter S, D’Armiento J, Henske EP, Arbiser ZK, Bai X, Goldberg G, Cohen C, Weiss SW (2002) Tuberous sclerosis-associated lesions of the kidney, brain and skin are angiogenic neoplasms. J Am Acad Dermatol 46:376–380PubMedCrossRefGoogle Scholar
  3. 3.
    Arita K, Kurisu K, Tominaga A, Sugiyama K, Eguchi K, Hama S, Yoshioka H, Yamasaki F, Kanou Y (2004) Relationship between intratumoral hemorrhage and overexpression of vascular endothelial growth factor (VEGF) in pituitary adenoma. Hiroshima J Med Sci 53:23–27PubMedGoogle Scholar
  4. 4.
    Barbosa-Coutinho LM, Lima EL, Gadret RO, Ferreira NP (1991) Hemorragia maciça intratumoral em esclerose tuberosa. Arq Neuropsiquiatr 49:465–470PubMedCrossRefGoogle Scholar
  5. 5.
    Buccoliero AM, Franchi A, Castiglione F, Gheri CF, Mussa F, Giordano F (2009) Subependymal giant cell astrocytoma (SEGA): is it an astrocytoma? Morphological, immunohistochemical and ultrastructural study. Neuropathology 29:25–30PubMedCrossRefGoogle Scholar
  6. 6.
    Cheng SY, Nagane M, Huang HS, Cavenee WK (1997) Intracerebral tumor-associated hemorrhage caused by overexpression of the vascular endothelial growth factor isofroms VEGF121 an VEGF165 but not VEGF189. Proc Natl Acad Sci USA 94:12081–12087PubMedCrossRefGoogle Scholar
  7. 7.
    Chow CW, Klug GL, Lewis EA (1988) Subependymal giant cell astrocytoma in children: an unusual discrepancy between histological and clinical features. J Neurosurg 68:880–883PubMedCrossRefGoogle Scholar
  8. 8.
    Cuccia V, Zuccaro G, Sosa F, Monges J, Lubieneky F, Taratuto A (2003) Subependymal giant cell astrocytoma in children with tuberous sclerosis. Child Nerv Syst 19:232–243Google Scholar
  9. 9.
    Frèrebeau P, Benezech J, Segnarbieux F, Harbi H, Desy A, Marty-Double C (1985) Intraventricular tumors in tuberous sclerosis. Childs Nerv Syst 1:45–48PubMedCrossRefGoogle Scholar
  10. 10.
    Gottfried ON, Fults DW, Townsend JT, Couldwell WT (2003) Spontaneous hemorrhage associated with a pilomyxoid astrocytoma: case report. J Neurosurg 99:416–420PubMedCrossRefGoogle Scholar
  11. 11.
    Hamamoto O, Honorato DC, Brito HLF, Souza-Queiroz L (1994) Intratumoral hemorrhage in tuberous sclerosis: case report. Arq Neuro-Psiquiatr 52:435–438CrossRefGoogle Scholar
  12. 12.
    Inamasu J, Nakamura Y, Saito R, Kuroshima Y, Mayanari K, Ischikizaki K (2005) Rebleeding from a primary brain tumor manifesting as intracerebral hemorrhage. Clin Neurol Neurosurg 108:105–108PubMedCrossRefGoogle Scholar
  13. 13.
    Jung S, Moon KS, Jung TY, Kim IY, Lee YH, Rhu HH, Sun HS, Jeong YI, Kim KK, Kang SS (2006) Possible pathophysiological role of vascular endothelial growth factor (VEGF) and matrix metalloproteinases (MMPs) in metastatic brain tumor-associated intracerebral hemorrhage. J Neurooncol 76:257–263PubMedCrossRefGoogle Scholar
  14. 14.
    Kalina P, Drehobi KE, Greenberg RW, Black KS, Hyman RA (1995) Hemorrhagic suependymal giant cell astrocytoma. Pedia Radiol 25:66–67CrossRefGoogle Scholar
  15. 15.
    Kato K, Moteki Y, Nakagawa M, Kadoyama S, Ujiie H (2011) Subaracnoid hemorrhage caused by pilocytic astrocytoma—case report. Neurol Med Chir 51:82–84CrossRefGoogle Scholar
  16. 16.
    Kim SK, Wang KC, Cho BK, Jung HW, Lee YJ, Chung YS, Lee JY, Park SH, KimYM CG, Chi JG (2001) Biological behavior and tumorigenesis of subependymal giant cell astrocytomas. J Neurooncol 52:217–225PubMedCrossRefGoogle Scholar
  17. 17.
    Kondziolka D, Bernstein M, Resch L, Tator CH, Flemming JFR, Vandelinden RG, Schutz H (1987) Significance of hemorrhage into brain tumors: clinicopathological study. J Neurosurg 67:852–857PubMedCrossRefGoogle Scholar
  18. 18.
    Kulmar R, Singh V (2004) Subependymal giant cell astrocytoma: a report of five cases. Neurosurg Rev 27:274–280Google Scholar
  19. 19.
    Kyrnetskiy EE, Kun LE, Boop FA, Sanford RA, Khan RB (2005) Types, causes, and outcome of intracranial hemorrhage in children with cancer. J Neurosurg 102(Suppl):31–35PubMedGoogle Scholar
  20. 20.
    Mahdavi A, Baradaran N, Nejat F, Khashab ME, Monajemzadeh M (2010) Sudden deterioration due to intra-tumoral hemorrhage of ependymoma of the fourth ventricle in a child during a flight: a case report. J Med - Case Rep 4:143–146PubMedCrossRefGoogle Scholar
  21. 21.
    Painter MJ, Pang D, Ahdab-Barmada M, Bergman I (1984) Connatal brain tumors in patients with tuberous sclerosis. Neurosurgery 14:570–573PubMedCrossRefGoogle Scholar
  22. 22.
    Romeike BF, Joellenbeck B, Skalej M, Scherlach C, Kirches E, Mawrin C (2007) Intraventricular meningioma with fatal haemorrhage: clinical and autopsy findings. Clin Neurol Neurosurg 109:884–887PubMedCrossRefGoogle Scholar
  23. 23.
    Salcman M (1992) Intracranial hemorrhage caused by brain tumor. In: Kaufman HH (ed) Intracerebral Hematomas. Raven, New York, pp 95–106Google Scholar
  24. 24.
    Scheithauer BW (1992) The neuropathology of tuberous sclerosis. J Dermatol 19:897–903PubMedGoogle Scholar
  25. 25.
    Stravinou P, Spiliotoupoulos A, Patsalas I, Balogiannis I, Karkavelas G, Polyzoidis K, Seeelviaridis P (2008) Subependymal giant cell astrocytoma with intratumoral hemorrhage in the absense of tuberous sclerosis. J Clin Neurosci 15:704–706CrossRefGoogle Scholar
  26. 26.
    Waga S, Yamamoto Y, Kojima T, Sakakura M (1977) Massive hemorrhage in tumor of tuberous sclerosis. Surg Neurol 8:99–101PubMedGoogle Scholar
  27. 27.
    Krueger DA, Care MM, Holland K, Agricola K, Tudor C, Mangeshkar P et al (2010) Everolimus for subependymal giant cell astrocytomas in tuberous sclerosis. N Engl Med 363:1801–1811CrossRefGoogle Scholar
  28. 28.
    Yalon M, Ben-Sira L, Constantini S, Toren A (2011) Regression of subependymal giant cell astrocytomas with RAD001 (Everolimus) in tuberous sclerosis complex. Childs Nerv Syst 27:179–181PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Hugo Sterman
    • 1
    • 2
  • Andre Beer Furlan
    • 1
    • 2
  • Hamilton Matushita
    • 1
    • 2
    • 4
  • Manoel Jacobsen Teixeira
    • 1
    • 3
  1. 1.Pediatric Neurosurgery Unit, Division of Neurosurgery, Hospital das ClínicasSão Paulo University Medical SchoolSão PauloBrazil
  2. 2.Pediatric NeurosurgerySão Paulo University Medical SchoolSão PauloBrazil
  3. 3.Department of NeurosurgerySão Paulo University Medical SchoolSão PauloBrazil
  4. 4.Bela VistaBrazil

Personalised recommendations