Article Figures & Data
Figures
- FIG 1.
Photographs of the patient demonstrate clinical sequelae of lipodystrophy, with profound loss of subcutaneous fat. Printed with authorization from the patient's parents.
- FIG 2.
Tumor appearance on axial MPRAGE (A) and T2WI (B), coronal postgadolinium T1-weighted Cube (GE Healthcare) (C), and sagittal FIESTA (D) and postgadolinium T1-weighted Cube (E). Images demonstrate an enhancing lobulated mass arising from the hypothalamic/chiasmatic region, with indistinct borders between the tumor and adjacent left hypothalamic parenchyma (arrow).
- FIG 3.
Leptomeningeal seeding seen on axial postgadolinium T1 Cube (A and B) and sagittal postgadolinium T1 images of the lower spine (C). Multiple enhancing foci are noted, including along the right temporal lobe (long straight arrow in A), ventral medulla (curved arrow in B), and ventral conus medullaris (short straight arrows in C). There is near-complete absence of subcutaneous fat, compatible with the patient's known lipodystrophy.
- FIG 4.
Photomicrographs of the tumor reveal a glioma composed of overall monotonous neoplastic cells embedded in a myxoid background (A, H&E, ×100 magnification) and exhibiting prominent angiocentric arrangements (B, H&E, ×200 magnification). Neoplastic cells demonstrate immunoreactivity for glial fibrillary acidic protein. C, Glial fibrillary acidic protein immunohistochemistry, ×200 magnification. Neurofilament reveals, overall, few background axons, compatible with a predominantly expansile growth pattern (D, neurofilament immunohistochemistry, ×100 magnification).
- FIG 5.
Artistic illustration of a PMA extending from the hypothalamus, with involvement of the suprasellar region and adjacent structures. PMAs tend to be solid and are typically devoid of both calcifications and intratumoral cysts. Used with permission of Mayo Foundation for Medical Education and Research. All rights reserved.
Tables
Comparison of typical PMA and pilocytic astrocytoma clinical and imaging features
PMA Pilocytic Astrocytoma Age Younger (mean age, 1.5 yr) Older (mean age, 4.8 yr) Location Hypothalamic/chiasmatic region Cerebellum > hypothalamic/chiasmatic region Enhancement Homogeneous Heterogeneous Intratumoral contents Most solid, with minimal tumoral cysts Most have cystic content Intratumoral hemorrhage 12%–25% 1%–8% Evidence of leptomeningeal seeding More frequent (up to 20%) Exceedingly rare
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