Hypothalamic Pilomyxoid Astrocytoma in a Child with Lipodystrophy

Pilomyxoid astrocytoma is a rare form of pediatric CNS malignancy first classified in 2007 by the World Health Organization. The tumors are similar to pilocytic astrocytomas, sharing both some imaging and histologic traits. However, pilomyxoid astrocytomas portend a more ominous prognosis, with more...

Full description

Saved in:
Bibliographic Details
Published in:American journal of neuroradiology : AJNR 2021-08, Vol.42 (8), p.1370-1374
Main Authors: Benson, J C, Trejo-Lopez, J, Bach, S E, Schwartz, J, Kaufmann, T J, Eckel, L, Guerin, J
Format: Article
Language:English
Subjects:
Astrocytoma - diagnostic imaging
Brain Neoplasms - diagnostic imaging
Child
Humans
Lipodystrophy
Magnetic Resonance Imaging
Pediatrics
Prognosis
Radiology-Pathology Correlation
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Pilomyxoid astrocytoma is a rare form of pediatric CNS malignancy first classified in 2007 by the World Health Organization. The tumors are similar to pilocytic astrocytomas, sharing both some imaging and histologic traits. However, pilomyxoid astrocytomas portend a more ominous prognosis, with more aggressive local tendencies and a greater proclivity for leptomeningeal spread. Although tissue sampling is ultimately required to differentiate pilocytic astrocytomas and pilomyxoid astrocytomas, some imaging features can be used to suggest a pilomyxoid astrocytoma, including homogeneous enhancement, leptomeningeal seeding, and lack of intratumoral cysts. In this article, a case of a hypothalamic pilomyxoid astrocytoma is described, in which the presenting disorder was profound generalized lipodystrophy. The aforementioned imaging characteristics of pilomyxoid astrocytomas are reviewed, as are the pathologic features of such tumors, including their angiocentric cellular arrangement and myxoid background.
ISSN:0195-6108
1936-959X
DOI:10.3174/ajnr.A7136