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Cerebral Edema ll: Pathophysiology01:22

Cerebral Edema ll: Pathophysiology

Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...

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Modeling Astrocytoma Pathogenesis In Vitro and In Vivo Using Cortical Astrocytes or Neural Stem Cells from Conditional, Genetically Engineered Mice
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Subependymal Giant Cell Astrocytomas in Tuberous Sclerosis Complex-Current Views on Their Pathogenesis and

Chao Gao1, Bernadeta Zabielska2, Fuyong Jiao3

  • 1Department of Rehabilitation Medicine, Henan Children's Hospital, Children's Hospital Affiliated to Zhengzhou University, Zhengzhou 450018, China.

Journal of Clinical Medicine
|February 11, 2023
PubMed
Summary

Tuberous sclerosis complex (TSC) treatment for subependymal giant cell astrocytoma (SEGA) has evolved. mTOR inhibitors offer a new therapeutic landscape, complementing or replacing traditional surgery for these benign brain tumors.

Keywords:
mTOR inhibitorsmanagementrecommendationssubependymal giant cell astrocytoma (SEGA)tuberous sclerosis complex (TSC)

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Area of Science:

  • Genetics and Oncology
  • Neurology

Background:

  • Tuberous sclerosis complex (TSC) is an autosomal-dominant disorder linked to TSC1/TSC2 gene mutations.
  • Subependymal giant cell astrocytomas (SEGAs) are slow-growing brain tumors predominantly found in TSC patients.
  • SEGAs, though benign, significantly contribute to morbidity and mortality in TSC children and adolescents.

Purpose of the Study:

  • To review the evolving therapeutic strategies for SEGA in TSC.
  • To compare the efficacy and limitations of mTOR inhibitors versus surgical resection for SEGA treatment.
  • To discuss the latest recommendations for SEGA management.

Main Methods:

  • Literature review of SEGA treatment modalities.
  • Analysis of the role of the mTOR pathway in SEGA development.
  • Discussion of clinical guidelines and recent research findings.

Main Results:

  • The discovery of the mTOR pathway's role has shifted SEGA treatment paradigms.
  • mTOR inhibitors present an alternative or adjunct to surgical resection.
  • Recent consensus guidelines propose an integrated approach to SEGA management.

Conclusions:

  • The therapeutic landscape for SEGAs in TSC has been transformed by mTOR inhibitors.
  • A balanced consideration of pharmacological and surgical options is crucial for optimal SEGA treatment.
  • The 2021 International Tuberous Sclerosis Complex Consensus Group recommendations offer a new framework for SEGA management.