Mass‐forming primary angiitis of central nervous system with <scp>R</scp>osai‐<scp>D</scp>orfmann disease‐like massive histiocytosis with emperipolesis

Kim, Seong Ik; Kim, Soo Hee; Cho, Hwa Jin; Kim, Hannah; Chung, Chong-Pyoung; Choi, Seung Hong; Park, Sung Sup

doi:10.1111/pin.12317

Cited by 7 publications

(3 citation statements)

References 19 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The solitary tumor-like mass lesions of PACNS are rare, which usually show high signal intensity on T2 and heterogeneously enhancing signals on Gd-enhanced images (17, 22–26). Previously reported mass-forming PACNS cases in adults from 2000 to the present are summarized in the Table 1.…”

Section: Discussionmentioning

confidence: 99%

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

Jin

Guo

et al. 2019

Front. Neurol.

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 99%

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

Jin

Guo

et al. 2019

Front. Neurol.

View full text Add to dashboard Cite

show abstract

“…Abnormal in ND 100% [18] 100% [12] ND ND 100% [47] 100% [19] 100% [31] 100% [5] 100% [ x Kumar, 2017 [30] x Takahashi, 2017 [31] x x x Lee, 2017 [32] x x Arif, 2017 [33] x x x Mizuno, 2016 [34] x x Sun, 2016 [35] x x x Benson, 2016 [36] x x Pillai, 2016 [37] x x x Fang, 2015 [38] x x x Kim, 2015 [39] x X x Bajaj, 2015 [40] x x x Kim SI, 2015 [41] x Killeen, 2015 [42] x x x Gan, 2015 [43] x x Huang, 2015 [44] x x x Okunomiya, 2014 [45] x x Safouris, 2014 [46] x x x Michiels, 2014 [47] x x x x Gaillard, 2014 [48] x x Rao, 2014 [49] x x Noh, 2014 [50] x x x Safouris, 2013 [46] x x x Orr, 2013 [51] x x x Lyra, 2013 [52] x x Rosenberg, 2013 [53] x x Okeda, 2012 [54] x x Yu, 2011 [55] x…”

Section: Black Blood Mrimentioning

confidence: 99%

“When should primary angiitis of the central nervous system (PACNS) be suspected?”: literature review and proposal of a preliminary screening algorithm

et al. 2020

View full text Add to dashboard Cite

Background Primary angiitis of the CNS (PACNS) is a process causing variously combined neurological disturbances. Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect. Objective (1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations. Methods Review of PUBMED case series on PACNS, published from 2002 to 2017, collection of frequencies of clinical and neuroimaging features and calculation of median values. Classification of features as “major” or “minor” if frequency was higher or lower than median value. Combination of features in sets of criteria represented by all possible combinations of major and minor clinical and neuroradiological features. Application of criteria to published PACNS case reports and selection of the ones best identifying patients with definite PACNS. Results We reviewed 24 case series. “Major” clinical features were headache, stroke, cognitive impairment, focal neurological deficits; “minor” were seizures, altered consciousness, psychiatric disorders. “Major” neuroradiological features were multiple parenchymal lesions, parenchymal/meningeal contrast enhancement, magnetic resonance angiography vessel abnormalities, vessel wall enhancement; “minor” were parenchymal/subarachnoid hemorrhage, single parenchymal lesion. The selected sets of criteria able to identify all PACNS patients were (1) one clinical (major/minor) + one major neuroradiological feature; and (2) Two clinical (≥ 1 major) + one minor neuroradiological feature. Conclusion Our review provides a detailed clinical/neuroradiological picture of PACNS. The proposed algorithm should be regarded as a preliminary screening tool to move the first steps towards PACNS diagnosis that needs validation.

show abstract

“… 40 However, Kim et al reported a case of PACNS that presented as a T2-hyperintense mass lesion, with some contrast enhancement on T1-weighted postcontrast imaging, in a 20-year-old woman presenting with seizures, highlighting the ability for PACNS to present in a similar manner to a primary CNS neoplasm. 33 A case series of four patients with PACNS-associated lesions reported by Lee et al demonstrated variable MRI findings, including inconsistent findings across DWI and PWI, in each patient, demonstrating the heterogeneity of the disease and the potential for diagnostic difficulties. 36 Similar mass lesions have been associated in other vasculopathies as well, including systemic lupus erythematosus, 41 and granulomatosis with polyangiitis has also been reported in the literature.…”

Section: Discussionmentioning

confidence: 95%

Pseudo-insular glioma syndrome: illustrative cases

Haddad¹,

Young²,

Morshed³

et al. 2021

Journal of Neurosurgery: Case Lessons

View full text Add to dashboard Cite

BACKGROUND Lower-grade insular gliomas often appear as expansile and infiltrative masses on magnetic resonance imaging (MRI). However, there are nonneoplastic lesions of the insula, such as demyelinating disease and vasculopathies, that can mimic insular gliomas. OBSERVATIONS The authors report two patients who presented with headaches and were found to have mass lesions concerning for lower-grade insular glioma based on MRI obtained at initial presentation. However, on the immediate preoperative MRI obtained a few weeks later, both patients had spontaneous and complete resolution of the insular lesions. LESSONS Tumor mimics should always be in the differential diagnosis of brain masses, including those involving the insula. The immediate preoperative MRI (within 24–48 hours of surgery) must be compared carefully with the initial presentation MRI to assess interval change that suggests tumor mimics to avoid unnecessary surgical intervention.

show abstract

Mass‐forming primary angiitis of central nervous system with Rosai‐Dorfmann disease‐like massive histiocytosis with emperipolesis

Cited by 7 publications

References 19 publications

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

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

“When should primary angiitis of the central nervous system (PACNS) be suspected?”: literature review and proposal of a preliminary screening algorithm

Pseudo-insular glioma syndrome: illustrative cases

Contact Info

Product

Resources

About