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Case Reports
. 2011 Aug 26:9:97.
doi: 10.1186/1477-7819-9-97.

Isolated angiitis of the central nervous system with tumor-like lesion, mimicking brain malignant glioma: a case report and review of the literature

Gan You  1 , Wei Yan, Wei Zhang, Shaowu Li, Guilin Li, Tao Jiang
Affiliations
Case Reports

Isolated angiitis of the central nervous system with tumor-like lesion, mimicking brain malignant glioma: a case report and review of the literature

Gan You et al. World J Surg Oncol. .

Abstract

Background: Isolated angiitis of the central nervous system (IACNS) is a rare but severe vascular disease, which could present like an isolated inflammatory lesion on magnetic resonance imaging (MRI). To date, only a few such cases with tumor-like IACNS have been reported.

Case presentation: A 35-year-old woman presented with headache and left-sided weakness. MRI scans initially mislead us to a diagnosis of glioblastoma (GBM). Surgery was performed. The mass was sub-totally resected. Pathological examination confirmed a cerebral vasculitis. Radiological features, such as disproportionate mass effect, striped hemorrhage and abnormal enhancement of adjacent vessels, could be helpful to distinguish a tumor-like IACNS from a GBM. Single therapy with high doses of steroid did not improve the patient's condition. Combined therapy with prednisolone and cyclophosphamide showed great benefit to the patient. No relapse occurred during the period of 18 months follow-up.

Conclusions: Although a tumor-like IACNS has no established imaging features, a diagnosis of tumor-like IACNS should be suspected when MRI shows inappropriate presentations of a tumor. Greater awareness of this potential manifestation of IACNS may facilitate more prompt diagnosis and treatment.

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Figures

Figure 1
Figure 1
Axial T2-weighted MR image (a) showing a mass with mixed signal intensity and a surrounding edema area. On the T1-weighted image after the administration of contrast material (b and c), the mass has an inhomogeneous enhancement. Sagittal T1-weighted MR image without contrast (d) depicting a striped hemorrhage.
Figure 2
Figure 2
Axial contrast-enhanced MR image showing a partial resection of the lesion. (a) T1-weighted image showing the newly developed hyper-intensity diffusing to the opposite side (b). Great improvement after the treatment (c). T1-weighted MR images with contrast showing some abnormally enhanced vessels (arrows) (d).
Figure 3
Figure 3
Pathologic findings showing massive perivascular lymphocyte cuffing (HE stain, × 200).
See this image and copyright information in PMC

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