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

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.

Tumefactive Primary Central Nervous System Vasculitis: Imaging Findings of a Rare and Underrecognized Neuroinflammatory Disease

“…[27][28][29][30] Our t-PCNSV cohort had a male predominance, which was observed in previous studies. 3,6,8,25 In our cohort, the most common presentations of patients with t-PCNSV were headaches, seizures, and focal neurologic deficits, which were similar to prior studies. [6][7][8]25 In our study, even though-PCNSV had a variety of imaging patterns, we could identify several imaging features shared by many of the patients with t-PCNSV, which may help raise the possible diagnosis of this disease.…”

“…Radiologic features of t-PCNSV were not well described or recognized because it is extremely rare entity. 4 To our knowledge, apart from several case reports [11][12][13][14][15][16][17][18][19][20][21][22] and review articles, [23][24][25] only a few small case series have been published in the medical literature. [6][7][8]10 Molloy et al 7 reported 8 cases of masslike PCNSV with the proved histopathology including both "Ab -related" PCNSV and "non-Ab -related" PCNSV from 2 tertiary institutions.…”

“…The most common location of t-PCNSV was the supratentorial cortex/subcortical areas, as shown in previous studies. 25 Deep gray nuclei involvement can be seen occasionally. Only 1 patient had brain stem involvement.…”

“…An underrecognized and rarer subset of PCNSV, approximately 5%-29%, can present with "masslike" or "tumefactive" lesions mimicking a neoplasm. 3,4,[6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] To date, imaging features of tumefactive PCNSV (t-PCNSV) have not been well described and definitive diagnosis can only be made confidently on histopathology. [5][6][7][8][9][10] In this article, we performed a retrospective review of 10 histopathologically proved cases of t-PCNSV and analyzed pertinent imaging features with histopathologic correlation.…”

Streptococcus Intermedius: A Mimicker of Brain Metastases and A Potential Pitfall for Radiation Oncologists

“…11 , 12 , 13 As with our patient, white blood cell count may be normal on initial presentation in patients with brain abscesses. Jin et al 14 described a case of a young female patient who developed neurologic symptoms in the setting of a peripherally enhancing brain lesion, initially thought to represent a tumor; however, biopsy revealed this mass to be caused by a primary angiitis of the central nervous system. Similarly, a young female patient with an intracranial mass mimicking a brain tumor was instead found to have an angiitis caused by systemic lupus erythematosus.…”