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.