Subependymal Giant Cell Astrocytoma: Associated Hyperproteinorrhachia Causing Shunt Failures and Nonobstructive Hydrocephalus - Report of Successful Treatment with Long-term Follow-up

Subependymal giant cell astrocytomas (SEGAs) are histologically benign tumors most frequently associated with tuberous sclerosis complex (TSC). Despite their benign histopathological appearance, they may cause unfavorable outcomes due to their intraventricular location. Rarely, SEGA may be associated with hyperproteinorrhachia (high levels of proteins in the cerebrospinal fluid [CSF]), which causes malresorptive, communicating hydrocephalus; certainly, this scenario makes shunt obstruction likely in this patient population. In this report, we illustrate the case of hyperproteinorrhachia in an SEGA patient with known TSC, who presented repeatedly with shunt failure from proteinaceous shunt obstruction. Subsequent surgical resection of the main intraventricular lesion resulted in a dramatic drop in the CSF protein levels and has since prevented further shunt failures. Different treatment concepts and possible pathophysiology are discussed and the pertinent literature is reviewed.

• Publication year

  2017

• Venue

  Asian Journal of Neurosurgery

• Publication date

  2017-10-01

• Fields of study

  Medicine

• Identifiers
  DOI 10.4103/ajns.AJNS_231_16PMID 29114301PMCID 5652113
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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