Hypothalamic pilomyxoid astrocytoma in a child with lipodystrophy

J. C. Benson, J. Trejo-Lopez, S. E. Bach, J. Schwartz, T. J. Kaufmann, Laurence Eckel, J. Guerin

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1370-1374
Number of pages5
JournalAmerican Journal of Neuroradiology
Volume42
Issue number8
DOIs
StatePublished - Aug 1 2021

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology

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