Subependymal giant cell astrocytoma: Associated hyperproteinorrhachia causing shunt failures and nonobstructive hydrocephalus - Report of successful treatment with long-term follow-up

Abstract

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.

Experience using mTOR inhibitors for subependymal giant cell astrocytoma in tuberous sclerosis complex at a single facility

“…Neuroradiological follow-up was performed every 1-3 years concept of SEGA from emergent hydrocephalus treatment by a single department to prophylactic treatment by an interdisciplinary team appears warranted. Some differences between SEGA with and without iICP, such as variations in immunohistochemical features, genetic differences, and anaplastic features [50][51][52] or protein-producing functions [53,54], may relate to brain compliance or osmotic pressure. However, this study could not address this question.…”

“…Some differences between SEGA with and without iICP, such as variations in immunohistochemical features, genetic differences, and anaplastic features [ 50 – 52 ] or protein-producing functions [ 53 , 54 ], may relate to brain compliance or osmotic pressure. However, this study could not address this question.…”

Complex Factors in Hydrocephalus Development in Tuberous Sclerosis Complex: A Case Report of Subependymal Giant Cell Astrocytoma

“…Studies have indicated that the mechanism by which SEGA causes hydrocephalus involves enlargement of the SEGA leading to blockage of the foramen of Monro and the subsequent development of hydrocephalus [ 3 - 5 ]. However, three case reports involving four patients have suggested that hyperosmolarity due to hyperproteinorrhachia causes non-obstructive hydrocephalus [ 6 - 8 ].…”