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Persistent communicating hydrocephalus in adult tuberous sclerosis patients: a possible therapeutic role for everolimus

  • Case Report - Brain Tumors
  • Published:
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Abstract

Subependymal giant cell tumor (SGCT) is a benign intraventricular tumor, usually located near the foramen of Monro. It is almost always associated with tuberous sclerosis complex (TSC). SGCTs may obstruct cerebrospinal fluid (CSF) pathways. Rarely, they may secrete a protein-rich exudate, causing communicating hydrocephalus. Surgery is indicated for symptomatic lesions or growing asymptomatic lesions. The operative approach to SGCT has shifted from simple shunt placement to a more aggressive approach, leading to early attempts at gross total resection. Recently, the mTOR inhibitor everolimus has been approved for treating SGCT. In this article, we present two cases of recurrent shunt malfunctions in adult TSC patients with protein-secreting SGCTs. We describe the complexity of treating such patients with an emphasis on the role mTOR inhibitors may have in their management. We also review the literature on surgical management of SGCT-related hydrocephalus.

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Conflict of interests

All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements) or nonfinancial interest (such as personal or professional relationships, affiliations, knowledge, or beliefs) in the subject matter or materials discussed in this manuscript.

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The patients whose cases are described in this report as well as their family members have consented to the submission of this paper to the journal “Acta Neurochirurgica.”

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Correspondence to Yosef Laviv.

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Laviv, Y., Jackson, S. & Rappaport, Z.H. Persistent communicating hydrocephalus in adult tuberous sclerosis patients: a possible therapeutic role for everolimus. Acta Neurochir 157, 241–245 (2015). https://doi.org/10.1007/s00701-014-2309-0

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  • DOI: https://doi.org/10.1007/s00701-014-2309-0

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