Subependymal Giant Cell Astrocytoma Associated with Tuberous Sclerosis Complex – Pharmacological Treatment using mTOR Inhibitors

Česká verzia

Authors: K. Zitterbart
Authors place of work: Klinika dětské onkologie LF MU a FN BrnoÚstav experimentální biologie, PřF MU, BrnoRegionální centrum aplikované molekulární onkologie, Masarykův onkologický ústav, Brno
Published in the journal: Klin Onkol 2014; 27(6): 401-405
Category: Přehled
doi: https://doi.org/10.14735/amko2014401

Summary

Tuberous sclerosis complex is a neurocutaneous syndrome that results from a germline mutation in TSC1 or TSC2 genes. The pathogenic activation of mTORC1 leads to the development of subependymal giant cell astrocytomas in patients with tuberous sclerosis complex. Blocking of the dysregulated pathway with mTOR inhibitors has the potential to reduce the volume of this low‑ grade brain tumor. This article reviews the current knowledge on the pharmacological treatment of subependymal giant cell astrocytomas. A long‑term follow‑up and early therapeutic intervention should lead to mortality and morbidity reduction and quality of life improvement in patients with tuberous sclerosis complex associated tumors.

Key words:
subependymal giant cell astrocytoma − tuberous sclerosis complex – mTOR – everolimus

This study was supported by the European Regional Development Fund as well as by the State Budget of the Czech Republic: project RECAMO CZ.1.05/2.1.00/03.0101 and the project CEB CZ.1.07/2.3.00/20.0183.

The author has received honoraria from Novartis for the lecture with the article-related topic.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Submitted:
18. 11. 2014

Accepted:
20. 11. 2014

Zdroje

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