Brain Tumor Res Treat.  2019 Oct;7(2):164-167. 10.14791/btrt.2019.7.e37.

A Case of Primary Central Nervous System Vasculitis That Worsened Despite Early Corticosteroid Therapy

• Yoon SY
• Park KS
• Park SH
• Park JY

• KMID: 2461193
• DOI: http://doi.org/10.14791/btrt.2019.7.e37

Abstract

Primary central nervous system vasculitis (PCNSV) is rare, and the diagnosis is difficult to make because of its variable radiologic expressions. Early corticosteroid therapy often is effective. Herein we report the case of a 56-year-old man who had a well-enhanced cystic mass with severe edema in the right frontal lobe, which was initially felt to be a malignancy. Histologic examination of tissue removed at craniotomy revealed that it was a PCNSV. Despite early administration of corticosteroids, a new lesion developed within 3 days. The lesions responded to treatment with cyclophosphamide and corticosteroid.

Keyword

Vasculitis; Cysts; Neoplasms; Glucocorticoids

MeSH Terms

Adrenal Cortex Hormones
Craniotomy
Cyclophosphamide
Diagnosis
Edema
Frontal Lobe
Glucocorticoids
Humans
Middle Aged
Vasculitis
Vasculitis, Central Nervous System*
Adrenal Cortex Hormones
Cyclophosphamide
Glucocorticoids

Figure

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  Fig. 1 Brain CT and MRI revealing a cystic mass-like lesion in the right frontal lobe. A: Brain CT reveals a 6-cm low-density lesion. B: Brain MRI illustrates the lesion with low signal intensity on T1-weighted image (WI). C: Fluid-fluid level of the lesion with upper high and lower low signal intensity is illustrated on T2-WI. D: Well-develop capsule is illustrated on enhanced MRI. E: Diffusion WI reveals no restriction.

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  Fig. 2 Brain CT on 3rd day after admission illustrating increased peritumoral edema and subfalcial herniation.

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  Fig. 3 Immediate postoperative MRI demonstrates no residual tumor at the primary site (A), but there was a new lesion with well enhancement surrounding the occipital horn and trigone of the left lateral ventricle (arrow) (B and C).

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  Fig. 4 Histological findings of vasculitis in the brain lesion. The hematoxylin-eosin stain demonstrated pseudocapsule (A, original magnification ×20) with prominant lymphocyte infiltration into small vessel wall (arrow) (B, original magnification ×200). Immunohistochemistry stains revealed positive for CD3 (C) and CD31 (D), and these markers are related to lymphocytes (original magnification ×200).

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