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RT Journal Article
SR Electronic
T1 Hypothalamic Pilomyxoid Astrocytoma in a Child with Lipodystrophy
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 1370
OP 1374
DO 10.3174/ajnr.A7136
VO 42
IS 8
A1 Benson, J.C.
A1 Trejo-Lopez, J.
A1 Bach, S.E.
A1 Schwartz, J.
A1 Kaufmann, T.J.
A1 Eckel, L.
A1 Guerin, J.
YR 2021
UL http://www.ajnr.org/content/42/8/1370.abstract
AB 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.PMApilomyxoid astrocytoma