Cite

Copy

Tap on and choose 'Add to Home Screen' to create a shortcut app

Tap on and choose 'Add to Home Screen/Install App' to create a shortcut app

Supratentorial Pleomorphic Xanthoastrocytomas in Children Homepage

This page was last updated on January 2nd, 2023

 

Authors

Ali Etemad-Sajadi, M.D.

Sandrine de Ribaupierre, M.D.

Benedict Rilliet, M.D.

Section Editor

Kristian Aquilina, M.D.

Editor in Chief

Rick Abbott, M.D.

Introduction

PXA is a rare tumor occurring in children and young adults. This tumor is usually located superficially in the supratentorial compartment, mostly in the temporal and parietal lobes. The main initial clinical presentation is seizures. PXA usually has an indolent course, with slow progression and benign behavior, although aggressive cases have been reported. The gold standard for treatment is total tumor resection, whenever possible.

Key Points

  • Heterogeneous, superficial mass on imaging: The radiological appearance of PXAs is characteristically a superficial supratentorial mass with heterogeneous contrast enhancement. The tumors might be nodulocystic or purely solid.
  • Pleomorphic cells with nuclear atypia: The histological characteristics are considered benign despite significant cellular pleomorphism and nuclear atypia. Xanthomatous astrocytes are a classical feature (cells containing intracellular lipid), as well as leptomeningeal involvement.
  • Preferred treatment total resection: Total resection is the goal with the best chance for cure.

Your donations keep us going

The ISPN Guide is free to use, but we rely on donations to fund our ongoing work and to maintain more than a thousand pages of information created to disseminate the most up-to-date knowledge in the field of paediatric neurosurgery.

By making a donation to The ISPN Guide you are also indirectly helping the many thousands of children around the world whose treatment depends on well-informed surgeons.

Please consider making a donation today.

Use the app

The ISPN Guide can be used as a standalone app, both on mobile devices and desktop computers. It’s quick and easy to use.

Fully featured

Free registration grants you full access to The Guide and host of featured designed to help further your own education.

Stay updated

The ISPN Guide continues to expand both in breadth and depth. Join our mailing list to stay up-to-date with our progress.