Diffusion histogram profiles predict molecular features of grade 4 in histologically lower-grade adult diffuse gliomas following WHO classification 2021.

OBJECTIVES: In the latest World Health Organization classification 2021, grade 4 adult diffuse gliomas can be diagnosed with several molecular features even without histological evidence of necrosis or microvascular proliferation. We aimed to explore whole tumor histogram-derived apparent diffusion coefficient (ADC) histogram profiles for differentiating between the presence (Mol-4) and absence (Mol-2/3) of grade 4 molecular features in histologically lower-grade gliomas. METHODS: Between June 2019 and October 2022, 184 adult patients with diffuse gliomas underwent MRI. After excluding 121 patients, 18 (median age, 64.5 [range, 37-84 years]) Mol-4 and 45 (median 40 [range, 18-73] years) Mol-2/3 patients with histologically lower-grade gliomas were enrolled. Whole tumor volume-of-interest-derived ADC histogram profiles were calculated and compared between the two groups. Stepwise logistic regression analysis with Akaike's information criterion using the ADC histogram profiles with p values < 0.01 and age at diagnosis was used to identify independent variables for predicting the Mol-4 group. RESULTS: The 90th percentile (p < 0.001), median (p < 0.001), mean (p < 0.001), 10th percentile (p = 0.014), and entropy (p < 0.001) of normalized ADC were lower, and kurtosis (p < 0.001) and skewness (p = 0.046) were higher in the Mol-4 group than in the Mol-2/3 group. Multivariate logistic regression analysis revealed that the entropy of normalized ADC and age at diagnosis were independent predictive parameters for the Mol-4 group with an area under the curve of 0.92. CONCLUSION: ADC histogram profiles may be promising preoperative imaging biomarkers to predict molecular grade 4 among histologically lower-grade adult diffuse gliomas. CLINICAL RELEVANCE STATEMENT: This study highlighted the diagnostic usefulness of ADC histogram profiles to differentiate histologically lower grade adult diffuse gliomas with the presence of molecular grade 4 features and those without. KEY POINTS: • ADC histogram profiles to predict molecular CNS WHO grade 4 status among histologically lower-grade adult diffuse gliomas were evaluated. • Entropy of ADC and age were independent predictive parameters for molecular grade 4 status. • ADC histogram analysis is useful for predicting molecular grade 4 among histologically lower-grade gliomas.

Neuroimaging Features of Cytokine-related Diseases.

Cytokines are small secreted proteins that have specific effects on cellular interactions and are crucial for functioning of the immune system. Cytokines are involved in almost all diseases, but as microscopic chemical compounds they cannot be visualized at imaging for obvious reasons. Several imaging manifestations have been well recognized owing to the development of cytokine therapies such as those with bevacizumab (antibody against vascular endothelial growth factor) and chimeric antigen receptor (CAR) T cells and the establishment of new disease concepts such as interferonopathy and cytokine release syndrome. For example, immune effector cell-associated neurotoxicity is the second most common form of toxicity after CAR T-cell therapy toxicity, and imaging is recommended to evaluate the severity. The emergence of COVID-19, which causes a cytokine storm, has profoundly impacted neuroimaging. The central nervous system is one of the systems that is most susceptible to cytokine storms, which are induced by the positive feedback of inflammatory cytokines. Cytokine storms cause several neurologic complications, including acute infarction, acute leukoencephalopathy, and catastrophic hemorrhage, leading to devastating neurologic outcomes. Imaging can be used to detect these abnormalities and describe their severity, and it may help distinguish mimics such as metabolic encephalopathy and cerebrovascular disease. Familiarity with the neuroimaging abnormalities caused by cytokine storms is beneficial for diagnosing such diseases and subsequently planning and initiating early treatment strategies. The authors outline the neuroimaging features of cytokine-related diseases, focusing on cytokine storms, neuroinflammatory and neurodegenerative diseases, cytokine-related tumors, and cytokine-related therapies, and describe an approach to diagnosing cytokine-related disease processes and their differentials. ©RSNA, 2024 Supplemental material is available for this article.

Deep learning reconstruction for high-resolution computed tomography images of the temporal bone: comparison with hybrid iterative reconstruction.

PURPOSE: We investigated whether the quality of high-resolution computed tomography (CT) images of the temporal bone improves with deep learning reconstruction (DLR) compared with hybrid iterative reconstruction (HIR). METHODS: This retrospective study enrolled 36 patients (15 men, 21 women; age, 53.9 ± 19.5 years) who had undergone high-resolution CT of the temporal bone. Axial and coronal images were reconstructed using DLR, HIR, and filtered back projection (FBP). In qualitative image analyses, two radiologists independently compared the DLR and HIR images with FBP in terms of depiction of structures, image noise, and overall quality, using a 5-point scale (5 = better than FBP, 1 = poorer than FBP) to evaluate image quality. The other two radiologists placed regions of interest on the tympanic cavity and measured the standard deviation of CT attenuation (i.e., quantitative image noise). Scores from the qualitative and quantitative analyses of the DLR and HIR images were compared using, respectively, the Wilcoxon signed-rank test and the paired t-test. RESULTS: Qualitative and quantitative image noise was significantly reduced in DLR images compared with HIR images (all comparisons, p ≤ 0.016). Depiction of the otic capsule, auditory ossicles, and tympanic membrane was significantly improved in DLR images compared with HIR images (both readers, p ≤ 0.003). Overall image quality was significantly superior in DLR images compared with HIR images (both readers, p < 0.001). CONCLUSION: Compared with HIR, DLR provided significantly better-quality high-resolution CT images of the temporal bone.

Diffuse peritoneal dissemination of intracranial pure germinoma via ventriculoperitoneal shunt.

Germinomas frequently cause hydrocephalus, and ventriculoperitoneal shunts (VPS) have been commonly used for their management. Although VPS can potentially serve as a route for peritoneal dissemination of germinomas, the abdominal imaging characteristics of this rare yet important complication remain unknown. In this article, we report the computed tomography imaging findings of diffuse peritoneal dissemination of intracranial germinoma.

Radiological features of thyroid-like low-grade nasopharyngeal papillary adenocarcinoma: case series and systematic review.

PURPOSE: To comprehensively summarize the clinical data and CT/MRI characteristics of thyroid-like low-grade nasopharyngeal papillary adenocarcinoma (TL-LGNPPA). METHODS: Twenty-seven lesions from 25 study articles identified through a systematic review and three lesions from our institution associated with TL-LGNPPA were evaluated. RESULTS: The mean age of the patients at diagnosis was 35.7 years, and the male-to-female ratio was nearly half. The chief complaint was nasal obstruction, followed by epistaxis. All patients underwent excision. None of the patients had neck nodes or distant metastases. All patients survived with no locoregional/distant recurrence during 3-93 months of follow-up. All lesions were located at the posterior edge of the nasal septum, attached to the nasopharyngeal parietal wall, and showed no laterality. The mean lesion diameter was 1.7 cm. The margins of lesions were well-defined and lobulated, followed by well-defined smooth margins. None of lesions were associated with parapharyngeal space or skull base destruction. All lesions were iso- and low-density on non-contrast CT. Adjacent skull base sclerosis was detected in 63.6% of lesions. High signal intensity on T2-weighted imaging and mostly iso-signal intensity on T1-weighted imaging compared to muscle tissue. Most lesions were heterogeneous and exhibited moderate contrast enhancement. Relatively large lesions (≥1.4 cm) tended to be more lobulated than smooth margins compared to relatively small lesions (<1.4 cm) (p = 0.016). CONCLUSION: We summarized the clinical and radiological features of TL-LGNPPA to facilitate accurate diagnosis and appropriate management.

Apparent diffusion coefficient for differentiation between extra-nodal lymphoma and squamous cell carcinoma in the head and neck: a systematic review and meta-analysis.

BACKGROUND: Radiological differentiation between extra-nodal lymphoma and squamous cell carcinoma in the head and neck is often difficult due to their similarities. PURPOSE: To evaluate the diagnostic benefit of apparent diffusion coefficient (ADC) calculated from diffusion-weighted imaging (DWI) in differentiating the two. MATERIAL AND METHODS: A systematic review was performed by searching the MEDLINE, Scopus, and Embase databases in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 statement. Forest plots and the pooled mean difference of ADC values were calculated to describe the relationship between extra-nodal lymphoma and squamous cell carcinoma in the head and neck. Heterogeneity among studies was evaluated using the Cochrane Q test and I2 statistic. RESULTS: The review identified eight studies with 440 patients (441 lesions) eligible for meta-analysis. Among all studies, the mean ADC values of squamous cell carcinoma was 0.88 × 10-3mm2/s and that of lymphoma was 0.64 × 10-3mm2/s. In the meta-analysis, the ADC value of lymphoma was significantly lower than that of squamous cell carcinoma (pooled mean difference = 0.235, 95% confidence interval [CI] = 0.168-0.302, P <0.0001). The Cochrane Q test (chi-square = 55.7, P <0.0001) and I2 statistic (I2 = 87.4%, 95% CI = 77.4-93.0%) revealed significant heterogeneity. CONCLUSION: This study highlights the value of quantitative assessment of ADC for objective and reliable differentiation between extra-nodal lymphoma and squamous cell carcinoma in the head and neck. Conclusions should be interpreted with caution due to heterogeneity in the study data.

Combined cytotoxic and immune-stimulatory gene therapy for primary adult high-grade glioma: a phase 1, first-in-human trial.

BACKGROUND: High-grade gliomas have a poor prognosis and do not respond well to treatment. Effective cancer immune responses depend on functional immune cells, which are typically absent from the brain. This study aimed to evaluate the safety and activity of two adenoviral vectors expressing HSV1-TK (Ad-hCMV-TK) and Flt3L (Ad-hCMV-Flt3L) in patients with high-grade glioma. METHODS: In this dose-finding, first-in-human trial, treatment-naive adults aged 18-75 years with newly identified high-grade glioma that was evaluated per immunotherapy response assessment in neuro-oncology criteria, and a Karnofsky Performance Status score of 70 or more, underwent maximal safe resection followed by injections of adenoviral vectors expressing HSV1-TK and Flt3L into the tumour bed. The study was conducted at the University of Michigan Medical School, Michigan Medicine (Ann Arbor, MI, USA). The study included six escalating doses of viral particles with starting doses of 1×1010 Ad-hCMV-TK viral particles and 1×109 Ad-hCMV-Flt3L viral particles (cohort A), and then 1×1011 Ad-hCMV-TK viral particles and 1×109 Ad-hCMV-Flt3L viral particles (cohort B), 1×1010 Ad-hCMV-TK viral particles and 1×1010 Ad-hCMV-Flt3L viral particles (cohort C), 1×1011 Ad-hCMV-TK viral particles and 1×1010 Ad-hCMV-Flt3L viral particles (cohort D), 1×1010 Ad-hCMV-TK viral particles and 1×1011 Ad-hCMV-Flt3L viral particles (cohort E), and 1×1011 Ad-hCMV-TK viral particles and 1×1011 Ad-hCMV-Flt3L viral particles (cohort F) following a 3+3 design. Two 1 mL tuberculin syringes were used to deliver freehand a mix of Ad-hCMV-TK and Ad-hCMV-Flt3L vectors into the walls of the resection cavity with a total injection of 2 mL distributed as 0·1 mL per site across 20 locations. Subsequently, patients received two 14-day courses of valacyclovir (2 g orally, three times per day) at 1-3 days and 10-12 weeks after vector administration and standad upfront chemoradiotherapy. The primary endpoint was the maximum tolerated dose of Ad-hCMV-Flt3L and Ad-hCMV-TK. Overall survival was a secondary endpoint. Recruitment is complete and the trial is finished. The trial is registered with ClinicalTrials.gov, NCT01811992. FINDINGS: Between April 8, 2014, and March 13, 2019, 21 patients were assessed for eligibility and 18 patients with high-grade glioma were enrolled and included in the analysis (three patients in each of the six dose cohorts); eight patients were female and ten were male. Neuropathological examination identified 14 (78%) patients with glioblastoma, three (17%) with gliosarcoma, and one (6%) with anaplastic ependymoma. The treatment was well-tolerated, and no dose-limiting toxicity was observed. The maximum tolerated dose was not reached. The most common serious grade 3-4 adverse events across all treatment groups were wound infection (four events in two patients) and thromboembolic events (five events in four patients). One death due to an adverse event (respiratory failure) occurred but was not related to study treatment. No treatment-related deaths occurred during the study. Median overall survival was 21·3 months (95% CI 11·1-26·1). INTERPRETATION: The combination of two adenoviral vectors demonstrated safety and feasibility in patients with high-grade glioma and warrants further investigation in a phase 1b/2 clinical trial. FUNDING: Funded in part by Phase One Foundation, Los Angeles, CA, The Board of Governors at Cedars-Sinai Medical Center, Los Angeles, CA, and The Rogel Cancer Center at The University of Michigan.

Quality assessment of routine brain imaging at 0.55 T: initial experience in a clinical workflow.

The purpose of this study was to assess the quality of clinical brain imaging in healthy subjects and patients on an FDA-approved commercial 0.55 T MRI scanner, and to provide information about the feasibility of using this scanner in a clinical workflow. In this IRB-approved study, brain examinations on the scanner were prospectively performed in 10 healthy subjects (February-April 2022) and retrospectively derived from 44 patients (February-July 2022). Images collected using the following pulse sequences were available for assessment: axial DWI (diffusion-weighted imaging), apparent diffusion coefficient maps, 2D axial fluid-attenuated inversion recovery images, axial susceptibility-weighted images (both magnitude and phase), sagittal T1 -weighted (T1w) Sampling Perfection with Application Optimized Contrast images, sagittal T1w MPRAGE (magnetization prepared rapid gradient echo) with contrast enhancement, axial T1w turbo spin echo (TSE) with and without contrast enhancement, and axial T2 -weighted TSE. Two readers retrospectively and independently evaluated image quality and specific anatomical features in a blinded fashion on a four-point Likert scale, with a score of 1 being unacceptable and 4 being excellent, and determined the ability to answer the clinical question in patients. For each category of image sequences, the mean, standard deviation, and percentage of unacceptable quality images (<2) were calculated. Acceptable (rating ≥ 2) image quality was achieved at 0.55 T in all sequences for patients and 85% of the sequences for healthy subjects. Radiologists were able to answer the clinical question in all patients scanned. In total, 50% of the sequences used in patients and about 60% of the sequences used in healthy subjects exhibited good (rating ≥ 3) image quality. Based on these findings, we conclude that diagnostic quality clinical brain images can be successfully collected on this commercial 0.55 T scanner, indicating that the routine brain imaging protocol may be deployed on this system in the clinical workflow.

Dural and Leptomeningeal Diseases: Anatomy, Causes, and Neuroimaging Findings.

Meningeal lesions can be caused by various conditions and pose diagnostic challenges. The authors review the anatomy of the meninges in the brain and spinal cord to provide a better understanding of the localization and extension of these diseases and summarize the clinical and imaging features of various conditions that cause dural and/or leptomeningeal enhancing lesions. These conditions include infectious meningitis (bacterial, tuberculous, viral, and fungal), autoimmune diseases (vasculitis, connective tissue diseases, autoimmune meningoencephalitis, Vogt-Koyanagi-Harada disease, neuro-Behçet syndrome, Susac syndrome, and sarcoidosis), primary and secondary tumors (meningioma, diffuse leptomeningeal glioneuronal tumor, melanocytic tumors, and lymphoma), tumorlike diseases (histiocytosis and immunoglobulin G4-related diseases), medication-induced diseases (immune-related adverse effects and posterior reversible encephalopathy syndrome), and other conditions (spontaneous intracranial hypotension, amyloidosis, and moyamoya disease). Although meningeal lesions may manifest with nonspecific imaging findings, correct diagnosis is important because the treatment strategy varies among these diseases. ©RSNA, 2023 Online supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article. Quiz questions for this article are available through the Online Learning Center.

Imaging Features of Ectopic Tissues and Their Complications: Embryologic and Anatomic Approach.

Ectopic tissue is an anatomic abnormality in which tissue develops in an area outside its normal location. It is primarily caused by abnormalities during the process of embryologic development. Although the majority of individuals with ectopic tissues remain asymptomatic, various symptoms and associated complications can occur. Failure in normal embryologic development leads to loss of normal physiologic function or may result in harmful functions such as ectopic hormonal secretion in the ectopic pituitary adenoma. Ectopic tissues may also frequently mimic tumors. For example, developmental abnormalities in the pharyngeal pouches may result in an ectopic parathyroid gland and ectopic thymus, both of which are frequently misdiagnosed as tumors. Adequate knowledge of embryology is essential for understanding the differential diagnoses of ectopic tissues and facilitating appropriate management. The authors summarize the embryologic development and pathogenesis of ectopic tissues by using illustrations to facilitate a deeper understanding of embryologic development and anatomy. Characteristic imaging findings (US, CT, MRI, and scintigraphy) are described for ectopic tissues of the brain, head, neck, thorax, abdomen, and pelvis by focusing on common conditions that radiologists may encounter in daily practice and their differential diagnoses. ©RSNA, 2023 Quiz questions for this article are available through the Online Learning Center.

Performance of axial diameter on MR imaging for identification of retropharyngeal lymph node metastases in head and neck cancer: a systematic review and meta-analysis.

PURPOSE: To summarize previous studies' data and to calculate the diagnostic performance of minimum axial diameter (MIAD) and maximum axial diameter (MAAD) on each of the cutoff values in retropharyngeal lymph node (RPLNs) metastases in head and neck cancer. METHODS: MEDLINE, Scopus, and Embase databases were searched for systematic review. Meta-analysis was performed to summarize estimates of sensitivity, specificity, and diagnostic odds ratio (DOR) and generate summary recipient operator characteristic (sROC). RESULTS: The review identified 5 studies with a total of 634 patients (971 lesions) that were eligible for the meta-analysis. The estimated sensitivity, specificity, and DOR at MIAD 5 mm cutoff and MIAD 6 mm cutoff were 89.8% and 74.3%, 82.7% and 92.7%, and 39.1 and 57.9, respectively. The estimated sensitivity, specificity, and DOR at MAAD 7 mm cutoff and MAAD 8 mm cutoff were 90.3% and 84.7%, 62.7% and 79.9%, and 17.8 and 21.7, respectively. The AUCs of sROC at MIAD 5 mm cutoff and MIAD 6 mm cutoff were 0.922 and 0.943. At MAAD 7 mm and MAAD 8 mm, they were 0.840 and 0.888. CONCLUSION: The diagnostic performance of the MIAD 6 mm cutoff in RPLN metastases from head and neck cancer was 2% higher than the MIAD 5 mm cutoff. The diagnostic performance of MIAD was higher than that of MAAD.

Dynamic contrast-enhanced MRI parameters and apparent diffusion coefficient as treatment response markers of skull base osteomyelitis: a preliminary study.

Purpose: Currently, there is no definitive consensus on the optimal imaging modality for determining the treatment response in patients with skull base osteomyelitis (SBO). This study aimed to investigate the utility of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) parameters and apparent diffusion coefficient (ADC) as treatment response markers of SBO. Material and methods: This study included 6 patients with SBO, who underwent both pre- and post-treatment DCE-MRI and diffusion-weighted imaging (DWI). Quantitative DCE-MRI parameters and ADC of the region-of-interest were analysed. These normalized parameters were calculated by dividing the region-of-interest by the reference region. The Wilcoxon signed rank test was used to compare these parameters between pre- and post-treatment periods. Results: The normalized fraction of the extravascular extracellular space (Ve) and ADC of the post-treatment status of SBO was significantly lower than those of pre-treatment measures (p = 0.03). The normalized fraction of blood plasma (Vp), normalized rate of transfer from the blood plasma into the extravascular extracellular space (Ktrans), and normalized backflow leakage of material from the extravascular extracellular space into the blood plasma (Kep) demonstrated no significant differences between pre- and post-treatment. Conclusions: DCE-MRI parameters Ve and ADC demonstrated a significant reduction when comparing measures across the pre- and post-treatment periods. These parameters may potentially serve as a valuable surrogate treatment response marker for SBO activity.

Dynamic susceptibility contrast-MRI parameters, ADC values, and the T2-FLAIR mismatch sign are useful to differentiate between H3-mutant and H3-wild-type high-grade midline glioma.

OBJECTIVES: Diffuse midline gliomas, H3K27-altered (DMG-A), are malignant gliomas with an unfavorable prognosis. Knowledge of dynamic susceptibility contrast (DSC) MRI findings and imaging differences with high-grade midline glioma without H3K27 alteration (DMG-W) has been limited. We compared the DSC, ADC, and conventional MRI findings between DMG-A and DMG-W. METHODS: In this single institutional retrospective study, the electronic database of our hospital between June 2015 and May 2021 was searched. Twenty and 17 patients with DMG-A (median, 13 years; range, 3-52 years; 11 females) and DMG-W (median, 40 years; 7-73 years; 9 females), respectively, were found. Normalized relative cerebral blood flow (nrCBF) and normalized corrected relative cerebral blood volume (ncrCBV); normalized maximum, mean, and minimum ADC values; and the prevalence of T2-FLAIR mismatch sign were compared between the two groups using Mann-Whitney U tests and Fisher's exact test. RESULTS: The nrCBF and ncrCBV were significantly lower in DMG-A compared with DMG-W (nrCBF: median 0.88 [range, 0.19-2.67] vs. 1.47 [range, 0.57-4.90] (p < 0.001); ncrCBV: 1.17 [0.20-2.67] vs. 1.56 [0.60-4.03] (p = 0.008)). Normalized maximum ADC (nADCmax) was significantly higher in DMG-A (median 2.37 [1.25-3.98] vs. 1.95 [1.23-2.77], p = 0.02). T2-FLAIR mismatch sign was significantly more common in DMG-A (11/20 (55.0%) vs. 1/17 (5.9%), p = 0.0017). When at least two of nrCBF < 1.11, nADCmax ≥ 2.48, and T2-FLAIR mismatch sign were positive, the diagnostic performance was the highest with accuracy of 0.81. CONCLUSION: DSC-MRI parameters, ADC values, and the T2-FLAIR mismatch sign are useful to differentiate between DMG-A and DMG-W. KEY POINTS: • Diffuse midline glioma, H3K27-altered (DMG-A), showed a significantly lower normalized relative cerebral blood flow and volume compared with H3K27-wild-type counterparts (DMG-W). • T2-fluid-attenuated inversion recovery (FLAIR) mismatch sign was significantly more frequent in DMG-A compared to DMG-W. • Indicators that combined DSC parameters, ADC values, and T2-FLAIR mismatch sign, with or without age, are useful to distinguish the two tumors.

Preoperative prediction for mastoid extension of middle ear cholesteatoma using temporal subtraction serial HRCT studies.

OBJECTIVES: This study investigated the utility of temporal subtraction computed tomography (TSCT) obtained with temporal bone high-resolution computed tomography (HRCT) for the preoperative prediction of mastoid extension of middle ear cholesteatomas. METHODS: Twenty-eight consecutive patients with surgically proven middle ear cholesteatomas were retrospectively evaluated. The presence of black color in the mastoid region on TSCT suggested progressive changes caused by bone erosion. Enlarged width of the anterior part of mastoid on HRCT was interpreted as suggestive of mastoid extension. Fisher's exact test was used to compare the widths and black color on TSCT for cases with and without mastoid extension. The diagnostic accuracy of TSCT and HRCT for detecting mastoid extension and interobserver agreement during the evaluation of black color on TSCT were calculated. RESULTS: There were 15 cases of surgically proven mastoid extension and 13 cases without mastoid extension. Patients with black color on TSCT were significantly more likely to have a mastoid extension (p < 0.001). The sensitivity and specificity of TSCT were 0.93 and 1.00, respectively. Patients in whom the width of the anterior part of the mastoid was enlarged were significantly more likely to have a mastoid extension (p = 0.007). The sensitivity and specificity of HRCT to detect the width of the anterior part of the mastoid were 0.80 and 0.77, respectively. Interobserver agreement during the evaluation of TSCT findings was good (k = 0.71). CONCLUSIONS: This novel TSCT technique and preoperative evaluations are useful for assessing mastoid extension of middle ear cholesteatomas and making treatment decisions. KEY POINTS: •TSCT shows a clear black color in the mastoid region when the middle ear cholesteatoma is accompanied by mastoid extension. •TSCT obtained with preoperative serial HRCT of the temporal bone is useful for assessing mastoid extension of middle ear cholesteatomas.

Central Nervous System Systemic Lupus Erythematosus: Pathophysiologic, Clinical, and Imaging Features.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by multiple immunologic abnormalities and has the potential to involve the central nervous system (CNS). The prevalence of SLE seems to be growing, possibly because of earlier diagnosis and improved survival; however, the associated mortality is still high. The mortality is associated with disease-related risk factors such as lupus disease activity, young age, and organ damage or with antiphospholipid syndrome (APS). Neuropsychiatric SLE (NPSLE), which is caused by SLE-related CNS involvement, comprises a broad range of neurologic and psychiatric manifestations with varying severity, which can make this disease indistinguishable from other conditions that are unrelated to SLE. No unifying pathophysiology has been found in the etiology of NPSLE, suggesting that this condition has multiple contributors such as various immune effectors and the brain-intrinsic neuroimmune interfaces that are breached by the immune effectors. The postulated neuroimmune interfaces include the blood-brain barrier, blood-cerebrospinal fluid barrier, meningeal barrier, and glymphatic system. On the basis of the immunologic, pathologic, and imaging features of NPSLE, the underlying pathophysiology can be classified as vasculitis and vasculopathy, APS, demyelinating syndrome, or autoimmune antibody-mediated encephalitis. Each pathophysiology has different imaging characteristics, although the imaging and pathophysiologic features may overlap. Moreover, there are complications due to the immunocompromised status caused by SLE per se or by SLE treatment. Radiologists and clinicians should become familiar with the underlying mechanisms, radiologic findings, and complications of NPSLE, as this information may aid in the diagnosis and treatment of NPSLE. Online supplemental material is available for this article. ©RSNA, 2022.

Deadly Fungi: Invasive Fungal Rhinosinusitis in the Head and Neck.

Invasive fungal rhinosinusitis (IFRS) is a serious infection that is associated with high morbidity and mortality rates. The incidence of IFRS has been increasing, mainly because of the increased use of antibiotics and immunosuppressive drugs. Rhino-orbital cerebral mucormycosis has recently reemerged among patients affected by COVID-19 and has become a global concern. The detection of extrasinus involvement in its early stage contributes to improved outcomes; therefore, imaging studies are essential in establishing the degree of involvement and managing the treatment properly, especially in immunocompromised patients. The common sites of extrasinus fungal invasion are the intraorbital, cavernous sinus, and intracranial regions. Fungi spread directly to these regions along the blood vessels or nerves, causing devastating complications such as optic nerve ischemia or compression, optic neuritis or perineuritis, orbital cellulitis, cavernous sinus thrombosis, mycotic aneurysm, vasculitis, internal carotid arterial occlusion, cerebral infarction, cerebritis, and brain abscess. IFRS has a broad imaging spectrum, and familiarity with intra- and extrasinonasal imaging features, such as loss of contrast enhancement of the affected region, which indicates tissue ischemia due to angioinvasion of fungi, and the surrounding anatomy is essential for prompt diagnosis and management. The authors summarize the epidemiology, etiology, risk factors, and complications of IFRS and review the anatomy and key diagnostic imaging features of IFRS beyond the sinonasal regions. ©RSNA, 2022.

Major Changes in 2021 World Health Organization Classification of Central Nervous System Tumors.

The World Health Organization (WHO) published the fifth edition of the WHO Classification of Tumors of the Central Nervous System (WHO CNS5) in 2021, as an update of the WHO central nervous system (CNS) classification system published in 2016. WHO CNS5 was drafted on the basis of recommendations from the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy (cIMPACT-NOW) and expounds the classification scheme of the previous edition, which emphasized the importance of genetic and molecular changes in the characteristics of CNS tumors. Multiple newly recognized tumor types, including those for which there is limited knowledge regarding neuroimaging features, are detailed in WHO CNS5. The authors describe the major changes introduced in WHO CNS5, including revisions to tumor nomenclature. For example, WHO grade IV tumors in the fourth edition are equivalent to CNS WHO grade 4 tumors in the fifth edition, and diffuse midline glioma, H3 K27M-mutant, is equivalent to midline glioma, H3 K27-altered. With regard to tumor typing, isocitrate dehydrogenase (IDH)-mutant glioblastoma has been modified to IDH-mutant astrocytoma. In tumor grading, IDH-mutant astrocytomas are now graded according to the presence or absence of homozygous CDKN2A/B deletion. Moreover, the molecular mechanisms of tumorigenesis, as well as the clinical characteristics and imaging features of the tumor types newly recognized in WHO CNS5, are summarized. Given that WHO CNS5 has become the foundation for daily practice, radiologists need to be familiar with this new edition of the WHO CNS tumor classification system. Online supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article. ©RSNA, 2022.

Neuroradiological features of the polymorphous low-grade neuroepithelial tumor of the young: five new cases with a systematic review of the literature.

PURPOSE: Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) is a newly recognized brain tumor with genetic abnormalities frequently involving either BRAF or FGFR2/FGFR3. There are few publications available about the neuroradiological features of PLNTY. In this systematic review, we assessed the demographic, clinical, and neuroradiological features of PLNTY. METHODS: Literature data were extracted from database searches in MEDLINE and SCOPUS databases up to June 10, 2021. Studies reporting on pathologically proven PLNTY with neuroradiological findings were included. After reviewing 103 abstracts, 9 articles encompassing 19 cases met the inclusion criteria. We also added five patients from our hospital. The correlations between the presence of "transmantle-like sign" and the following three factors: duration of seizures; tumor size; and pathologically proven cortical dysplasia, were examined. RESULTS: The median patient age was 15.5 years (range, 5-57 years), and 15/24 (62.5%) were female. All tumors were localized supratentorialy. The main radiological features included cortical or subcortical masses (95.8%) in the temporal lobe (66.7%), calcification (83.3%), well-defined margins (72.7%), solid and cystic components (66.6%), and T2-weighted imaging (T2WI) hyperintensity (50.0%). The duration of seizure was significantly longer (positive vs. negative (median [range]), 24 months [6 - 96 months] vs. 5 months [1 - 12 months], p = 0.042), and the presence of the cortical dysplasia was significantly more frequent (3/8 vs 0/16, p = 0.042) in the patients with transmantle-like sign. CONCLUSION: PLNTY typically represents a calcified, well-defined mass in the supratentorial cortical or subcortical regions. The radiological findings defined here could facilitate the diagnosis of PLNTY.

Comprehensive radiological features of laryngeal sarcoidosis: cases series and systematic review.

PURPOSE: To comprehensively summarize the characteristic radiological findings of laryngeal sarcoidosis. METHODS: We reviewed patients with laryngeal sarcoidosis who underwent computed tomography (CT) and/or magnetic resonance imaging (MRI) and included 8 cases from 8 publications that were found through a systematic review and 6 cases from our institutions. Two board-certified radiologists reviewed and evaluated the radiological images. RESULTS: Almost all cases exhibited supraglottic lesions 13/14 (92.9%) and most of them involved aryepiglottic folds (12/13, 92.3%), epiglottis (11/14, 78.6%), and arytenoid region (10/14, 71.4%). Most lesions were bilateral (12/14, 85.7%). All cases showed well-defined margins and a diffuse swelling appearance (14/14, 100%). Non-contrast CT revealed a low density (4/5, 80%). The contrast-enhanced CT showed a slight patchy enhancement predominantly at the margin of the lesion in most cases (12/13, 92.3%). In one case, T2-weighted images showed high signal intensity peripherally and low signal intensity centrally (1/1, 100%). Gadolinium-enhanced MRI showed moderate heterogeneous enhancement predominantly at the margin of the lesion (2/2, 100%). In one case, diffusion-weighted imaging showed intermediate signal intensity; the apparent diffusion coefficient value was 2.4 × 10-3 mm2/s. The larynx was the only region affected by sarcoidosis in 57.1% (8/14) of the cases. Involvement of the neck lymph nodes and distant organs was observed in 4/14 (28.6%) patients, respectively. CONCLUSION: We summarized the CT and MRI findings of patients with laryngeal sarcoidosis. Knowledge of these characteristics is expected to facilitate prompt diagnosis and appropriate management.

Radiological features of human papillomavirus-related multiphenotypic sinonasal carcinoma: systematic review and case series.

PURPOSE: To comprehensively summarize the radiological characteristics of human papillomavirus (HPV)-related multiphenotypic sinonasal carcinomas (HMSCs). METHODS: We reviewed the findings for patients with HMSCs who underwent computed tomography (CT) and/or magnetic resonance imaging (MRI) and included nine cases from nine publications that were identified through a systematic review and three cases from our institution. Two board-certified radiologists reviewed and evaluated the radiological images. RESULTS: The locations in almost all cases included the nasal cavity (11/12, 91.7%). The involved paranasal sinuses included the ethmoid sinus (6/12, 50.0%) and maxillary sinus (3/12, 25.0%). The mean long diameter of the tumors was 46.3 mm. The margins in 91.7% (11/12) of the cases were well-defined and smooth. Heterogeneous enhancement on contrast-enhanced CT, heterogeneous high signal intensities on T2-weighted images and heterogeneous enhancement on gadolinium-enhanced T1-weighted images were noted in 2/2, 5/5, and 8/8 cases, respectively. Mean apparent diffusion coefficient values in two cases of our institution were 1.17 and 1.09 × 10-3 mm2/s. Compressive changes in the surrounding structures were common (75%, 9/12). Few cases showed intraorbital or intracranial extension. None of the cases showed a perineural spread, neck lymph node metastasis, or remote lesions. CONCLUSIONS: We summarized the CT and MRI findings of HMSCs. Knowledge of such characteristics is expected to facilitate prompt diagnosis and appropriate management.