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Neurofibromatosis Type I - Bone Dysplasia
Joseph Junewick, MD FACR
over 7 years ago
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Case History


13 year old female with headaches.


Case Detail

Anatomy: Brain-Spine
Borders
Heather Borders, MD
Diagnostic Category: Neoplasia Benign
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: MRI
Activities:
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Reveal Findings

Large cystic mass with an enhancing peripheral nodule. Nodule is isointense to gray matter on T1 and T2. Cystic portion is isointense to CSF. No significant surrounding edema. Mild midline shift and mass effect.

Reveal Discussion

Pleomorphic xanthoastrocytoma is a rare, usually benign, cortical-based mass that often enhances intensely with contrast material. Typically a peripheral mass with a cortically based nodule.

The most common location is in the temporal lobes (Temporal>parietal>frontal). Nearly all are supratentorial. Seizures and headaches are common clinical features. Familiarity with this lesion is important in the differential diagnosis of enhancing cortical-based masses.

Differential diagnosis includes, ganglioglioma, JPA, low grade glioma, oligodendroglioma.

MRI findings:
T1=nodule isointense to gray matter or mildly heterogeneous, cystic portion is isointense to CSF
T2=nodule hyperintense to minimally heterogeneous, usually an absence of surrounding edema, cystic portion is isointense to CSF
Post-contrast=intense enhancement of the nodule, possibly adjacent meningeal enhancement +/-dural tail

Treatment is surgical. Recurrence is rare, but if it happens, repeat surgery is the treatment.

Reveal Diagnosis

Pleomorphic xanthoastrocytoma

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