Case 14216

Pleomorphic xanthoastrocytoma
with dural involvement
Published on 14.12.2016

DOI: 10.1594/EURORAD/CASE.14216
ISSN: 1563-4086
Section: Neuroradiology
Area of Interest: Neuroradiology brain Paediatric
Procedure: Diagnostic procedure
Imaging Technique: MR
Imaging Technique: MR-Diffusion/Perfusion
Special Focus: Neoplasia Case Type: Clinical Cases
Authors: Pablo Naval-Baudin1, Marta Gómez Chiari2,
Jordi Muchart2, Mónica Rebollo2
Patient: 13 months, female

Clinical History:

A 13-month-old female infant is brought to consultation due to repeated spastic seizures during the 2 previous
months, without loss of consciousness, which have progressively become more frequent, now occurring up to 5-6
times a day. The patient is initiated on anti-epileptic drugs and a cranial MRI is performed.
Imaging Findings:

Initial MRI (figure 1) demonstrates a cystic lesion located in the left anterior temporal pole with slight T2/FLAIR
hyperintensity of the adjacent parenchyma. No contrast enhancement is visible. The lesion is considered suggestive
of a low-grade glioma or ganglioglioma.
The patient is followed with consecutive, yearly MRI scans, without significant change in between.

Follow-up MRI performed 4 years later (figure 2) identifies the same lesion, of similar size, but which now shows a
large, intensely-enhancing extraaxial component in the adjacent skull-base with fine dural tail. This extraaxial
component, which represents meningeal involvement is highly suggestive of pleomorphic xanthoastrocytoma.

The tumour is resected (left temporal lobectomy) and pathology confirms a pleomorphic xanthoastrocytoma with
anaplastic characteristics and leptomeningeal infiltration.

Seizures are correctly controlled after surgery and anticonvulsant drugs withdrawn.
Discussion:

A.-Background and Clinical Perspective

Pleomorphic xanthoastrocytoma (PXA) is a type of WHO grade II glial tumour. It is rare, comprising less than 1% of
all brain tumours and affects mainly children and young adults [1, 2].

The tumour is supratentorial in up to 98% of cases and frequently involves cortex and meninges [2].

Seizures are the most common form of presentation, occurring in most cases. Other initial symptoms include
headache, nausea and dizziness [3].

B.-Imaging Perspective
PXA are almost always situated supratentorially (>90%), most commonly in the temporal lobe (40-50%) followed by
frontal and parietal lobes [3].

These tumours frequently have a cystic component isointense to CSF (50-60%), and are located in cerebral
hemispheres with involvement of meninges. The solid component is usually hyperintense in T2 and FLAIR,
enhances intensely and homogenously in CT and MRI, and may show diffusion restriction [1, 2, 4]. Reaction of the
adjacent meninges forming a typical "dural tail" is fairly specific and may be a useful discriminating feature [4].

C.-Differential Diagnosis
- Ganglioglioma: Very similar appearance and location. It often calcifies and does not show dural reaction.
- Pilocytic astrocytoma: Most commonly infratentorial (especially in children).
- DNET: May calcify. Slow-growing lesion often abuts and remodels calvarium.
- Oligodendroglioma: Gross calcifications. Usually involves cortex (with signal change and thickening).
- Meningioangiomatosis: cortical/subcortical mass with gross calcification and enhancing meningovascular
proliferation.

D.-Outcome
PXA is usually circumscribed and slow-growing. Surgery is the treatment of choice, and recurrence is uncommon
with 70% survival at 10 years.

E.-Teaching Points
- Supratentorial (temporal) cyst with an intensely enhancing mural nodule.
- Child or young adult with long standing epilepsy, most common presentation
- Often difficult to differentiate from other cystic-nodular supratentorial lesions. Identification of an enhancing dural
tail may be a key discriminating factor.
Differential Diagnosis List: Pleomorphic xanthoastrocytoma with dural reaction., Ganglioglioma, Pilocytic
astrocytoma, DNET, Oligodendroglyoma, Meningioangiomatosis

Final Diagnosis: Pleomorphic xanthoastrocytoma with dural reaction.

References:

Moore W, Mathis D, Gargan L, Bowers DC, Klesse LJ, Margraf L, Koral K (2014) Pleomorphic xanthoastrocytoma of
childhood: MR imaging and diffusion MR imaging features. AJNR Am J Neuroradiol Nov-Dec;35(11):2192-6 (PMID:
24994821)
Plaza MJ, Borja MJ, Altman N, Saigal G (2013) Conventional and advanced MRI features of pediatric intracranial
tumors: posterior fossa and suprasellar tumors. AJR Am J Roentgenol May;200(5):1115-24 (PMID: 23617498)
Ida CM, Rodriguez FJ, Burger PC, Caron AA, Jenkins SM, Spears GM, Aranguren DL, Lachance DH, Giannini C
(2015) Pleomorphic Xanthoastrocytoma: Natural History and Long-Term Follow-Up. Brain Pathol Sep;25(5):575-86
(PMID: 25318587)
Crespo-Rodríguez AM, Smirniotopoulos JG, Rushing EJ (2007) MR and CT imaging of 24 pleomorphic
xanthoastrocytomas (PXA) and a review of the literature. Neuroradiology Apr;49(4):307-15 (PMID: 17205313)
Figure 1
a

Description: T1-weighted sagittal image shows hypointense (cystic) lesion in anterior temporal pole.
Origin: Department of Radiology. Hospital Universitari Sant Joan de Deu.
b

Description: T2-weighted axial image shows hyperintense (cystic) lesion in anterior temporal pole, with
hyperintensity of adjacent parenchyma. Origin: Department of Radiology. Hospital Universitari Sant
Joan de Deu.
c

Description: FLAIR-weighted axial image shows hypointense (cystic) lesion in anterior temporal pole,
with hyperintensity of adjacent parenchyma. Origin: Department of Radiology. Hospital Universitari
Sant Joan de Deu.
d

Description: DWI shows mild to insignificant diffusion restriction on the wall of the cystic lesion.Origin:
Department of Radiology. Hospital Universitari Sant Joan de Deu.
e

Description: Coronal T1-weighted contrast-enhanced images show no anomalous enhancing

component. Origin: Department of Radiology. Hospital Universitari Sant Joan de Deu.
Figure 2
a

Description: Contrast-enhanced (2a), and non contrast-enhanced T1 (2b), T2 (2c) and FLAIR (2d).
The same left temporal anterior-pole lesion persists, now showing adjacent intensely-enhancing
meningeal thickening (arrows) with dural tail (arrowheads). Origin: Department of Radiology. Hospital
Universitari Sant Joan de Deu. Barcelona, Spain.
b

Description: Contrast-enhanced (2a), and non contrast-enhanced T1 (2b), T2 (2c) and FLAIR (2d).
The same left temporal anterior-pole lesion persists, now showing adjacent intensely-enhancing
meningeal thickening (arrows) with dural tail (arrowheads). Origin: Department of Radiology. Hospital
Universitari Sant Joan de Deu. Barcelona, Spain.
c

Description: Contrast-enhanced (2a), and non contrast-enhanced T1 (2b), T2 (2c) and FLAIR (2d).
The same left temporal anterior-pole lesion persists, now showing adjacent intensely-enhancing
meningeal thickening (arrows) with dural tail (arrowheads). Origin: Department of Radiology. Hospital
Universitari Sant Joan de Deu. Barcelona, Spain.
d

Description: Contrast-enhanced (2a), and non contrast-enhanced T1 (2b), T2 (2c) and FLAIR (2d).
The same left temporal anterior-pole lesion persists, now showing adjacent intensely-enhancing
meningeal thickening (arrows) with dural tail (arrowheads). Origin: Department of Radiology. Hospital
Universitari Sant Joan de Deu. Barcelona, Spain.