Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review

Hang Jin,Yang Qu,Zhen-Ni Guo,Fu-Liang Zhang,Yi Yang,Guo-Zhen Cui

doi:10.3389/fneur.2019.01208

Abstract

Primary angiitis of the central nervous system (PACNS) is a rare disorder resulting in idiopathic inflammation affecting the parenchymal and leptomeningeal vessels confined to the central nervous system (CNS), of which a tumor-like mass lesion is an even rare subtype. We described a case of PACNS initially misdiagnosed as glioblastoma. The patient was a 35 year-old female with right-sided weakness and expressive dysphasia. Brain MRI showed a tumor-like lesion highly suggestive of glioblastoma, therefor surgical removal was done. After a resection and an exhaustive workup, PACNS was ultimately diagnosed. The case illustrates a type of imaging presentation of PACNS that is often misdiagnosed as high-grade glioma. Differentiation between tumor-like PACNS lesions and actual CNS tumors is challenging due to similar MR images. To avoid unnecessary surgical interventions, we summarized previously reported mass-forming PACNS cases in adults from January 1, 2000, to December 31, 2018 and the imaging characteristics of PACNS. Some less commonly used diagnostic methods such as MR spectroscopy may also help clinicians distinguish PACNS from its mimics.

Highlights

Tumor-like mass lesion is a rare presentation of Primary angiitis of the central nervous system (PACNS), accounting for only 5% of all PACNS
This report describes a peculiar case of histopathologically proven PACNS presenting a tumor-like lesion, which was initially misdiagnosed as malignant glioma, and surgical removal was done unnecessarily
This study summarizes the imaging characteristics of tumor-like mass lesion PACNS to possibly avoid unnecessary surgical interventions

Summary

BACKGROUND

Tumor-like mass lesion is a rare presentation of PACNS, accounting for only 5% of all PACNS. This report describes a patient presenting with a cerebral mass lesion in the left parietal lobe, clinically consistent on initial neuroimaging with high-grade glioma, which was diagnosed of PACNS by histopathology. PACNS cases with tumor-like lesion similar to the current patient are rare. This report summarizes these cases from January 1, 2000, to December 31, 2018. A 35 year-old Chinese woman was admitted to our hospital 2 days after an acute onset of right-sided weakness and expressive dysphasia She had no history of infection and vaccination within 6 weeks. Tumor-Like Mass Lesion PACNS and no other medical history. A brain MRI showed a tumor-like lesion with surrounding edema in subcortical of the left parietal lobe highly suggestive of glioma. At a 1 year follow-up, the patient could walk independently

Findings

DISCUSSION

CONCLUSIONS