Solitary fibrous tumour of the dura

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Headache.

Patient Data

Age: 35 years
Gender: Female
This study is a stack
Axial
T2
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Axial
FLAIR
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Axial
T1
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Axial
T1 C+
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Coronal
T1
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Coronal
T1 C+
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Axial
DWI
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Axial
ADC
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Axial
SWI
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Axial SWI
Phase
This study is a stack
Axial MR
Perfusion (CBV)
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Info

A large multilobulated extra-axial mass in the left frontal region is isointense on T1 and T2 weighted images to the adjacent cortex and has vivid contrast enhancement.

The posterior component demonstrates diffusion restriction with ADC values similar to brain (~750 x 10-6 mm2/s) whilst the anterior component of the mass predominantly demonstrates facilitated diffusion, although there are tiny patchy areas of diffusion restriction at its centre. This implies differing cellularity.

Prominent vascularity is seen within the lesion. MR perfusion demonstrates significantly increased cerebral blood volume.

Surrounding hyperintense FLAIR signal in keeping with cerebral oedema. Mass effect with effacement of the left lateral ventricle and rightward midline shift of 5mm. Mild/early left uncal herniation. No tonsillar herniation. No hydrocephalus. No other abnormal lesions.

Conclusion

Large enhancing vascular extra-axial left frontal tumour, likely a meningioma, although there are multiple atypical features e.g. marked lobulation and vascularity over the surface of the mass. Variant/higher grade meningioma or solitary fibrous tumour are possible differentials.

Case Discussion

The patient went on to have a biopsy.

Histology

Sections show a highly cellular tumour attached to dura. The tumour is composed of a relatively uniform proliferation of ovoid spindle cells in haphazard fascicles, with intervening thin walled staghorn-shaped vessels. There are focal areas of lower cellularity, with interspersed dense keloidal-like collagen. Mitoses are relatively brisk, up to 6 per 10 HPF (2.5/mm2). There is no necrosis.

Immunohistochemistry

  • STAT6: Moderate to strong patchy nuclear positivity

  • CD34: Diffuse membranous positivity

  • EMA: Negative SSTR2: Negative

  • GFAP; Negative

  • Ki-67: Up to 20% in hotspots.

Final diagnosis: solitary fibrous tumour, CNS WHO grade 2.

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