gms | German Medical Science

60th Annual Meeting of the German Society of Neurosurgery (DGNC)
Joint Meeting with the Benelux countries and Bulgaria

German Society of Neurosurgery (DGNC)

24 - 27 May 2009, Münster

Neuroendoscopic management and cytogenetical analysis of subependymal giant cell astrocytoma in a twenty-seven-year-old patient with Bourneville syndrome

Meeting Abstract

  • E. Naydenov - Department of Neurosurgery, University Hospital "St. Ivan Rilsky", Sofia, Bulgaria
  • C. Tzekov - Department of Neurosurgery, University Hospital "St. Ivan Rilsky", Sofia, Bulgaria
  • K. Minkin - Department of Neurosurgery, University Hospital "St. Ivan Rilsky", Sofia, Bulgaria
  • A. Bussarsky - Department of Neurosurgery, University Hospital "St. Ivan Rilsky", Sofia, Bulgaria
  • R. Isakova - Department of Neurosurgery, University Hospital "St. Ivan Rilsky", Sofia, Bulgaria
  • O. Kalev - Department of Pathology, Medical University, Sofia, Bulgaria
  • S. Hadjidekova - Department of Medical Genetics, Medical University, Sofia, Bulgaria
  • D. Toncheva - Department of Medical Genetics, Medical University, Sofia, Bulgaria

Deutsche Gesellschaft für Neurochirurgie. 60. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit den Benelux-Ländern und Bulgarien. Münster, 24.-27.05.2009. Düsseldorf: German Medical Science GMS Publishing House; 2009. DocP12-06

DOI: 10.3205/09dgnc380, URN: urn:nbn:de:0183-09dgnc3806

Published: May 20, 2009

© 2009 Naydenov et al.
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Outline

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Objective: Bourneville syndrome (tuberous sclerosis complex – TSC) is a rare disease with autosomal dominant mode of inheritance. The condition is characterized by the potential for hamartoma formation in almost every organ. The presence of subependymal giant cell astrocytomas is seen in about 5% of the cases with TSC and in most of them does not necessitate neurosurgical intervention.

Results: A twenty-seven-year-old male patient was admitted to the clinic with a one-month-history of single generalized seizures. Electroencephalography showed paroxysmal activity in the right temporo-occipital region. Multiple periventricular and cortical lesions were found in neuroimaging studies (i.e. computed tomography and magnetic resonance imaging). Two types of dermatologic lesions were found during patient’s examination – hypomelanotic macules (one in the abdominal region and another on the right arm), as well as malar area facial angiofibromas. No neurological abnormalities were found. The abdominal sonography found hyperechogenic areas within the renal and hepatic parenchyma. Prophylactic septum pellucidum fenestration and partial excision of the periventricular lesion were performed using neuroendoscopic approach. The histological result was subependymal giant cell astrocytoma. A comparative genomic hybridization analysis was also performed using tumor sample. The patient recovered well after the intervention without any additional complaints.

Conclusions: Most of the patients having subependymal giant cell astrocytoma as a CNS-form of Bourneville syndrome do not need surgical intervention. The neuroendoscopic approach is a safe, minimally invasive, and effective method for obstructive hydrocephalus treatment in these cases.