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.

Post-treatment magnetic resonance imaging predicts outcomes of maxillary sinus cancer treatment using super-selective intra-arterial infusion of high-dose cisplatin with concomitant radiotherapy (RADPLAT).

OBJECTIVES: This study aimed to evaluate the prognostic value of magnetic resonance imaging (MRI) findings in predicting local recurrence in patients with maxillary sinus cancer treated with super-selective intra-arterial infusion of high-dose cisplatin with concomitant radiotherapy (RADPLAT). METHODS: This single-center retrospective study included consecutive patients with maxillary sinus squamous cell carcinoma, who underwent RADPLAT between October 2016 and September 2021. MRI was performed before (within 2 weeks) and 1 month after (post-treatment MRI) the start of treatment. Tumor reduction rates and pre-treatment cross-sectional areas were calculated from the maximum cross-sectional areas on pre- and post-treatment MRI T2-weighted axial images. Statistical analyses, including receiver operating characteristic analysis, were performed to assess the predictive value of the tumor reduction rates. RESULTS: Twenty-four patients were included in this study. Recurrence occurred in seven patients with a median time of 213 days. The tumor reduction rates were significantly higher in the benign post-treatment changes group compared to the recurrence group (median, 0.814 vs. 0.174; p < 0.001). The cut-off value for the reduction rate between the groups was 0.3578. No significant difference was observed in the maximum pre-treatment cross-sectional area between the groups (p = 0.664). The inter-observer agreement for the tumor areas was excellent. CONCLUSIONS: The tumor reduction rate calculated from MRI T2-weighted images may be a predictor of local recurrence in patients with maxillary sinus cancer treated with RADPLAT. Patients with lower reduction rates may benefit from early salvage surgeries.

Radiological Imaging Findings of Adrenal Abnormalities in TAFRO Syndrome: A Systematic Review.

This systematic review article aims to investigate the clinical and radiological imaging characteristics of adrenal abnormalities in patients with thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome. We searched the literature in PubMed, the Cochrane Library, and the Web of Science Core Collection. Ultimately, we analyzed 11 studies with 22 patients plus our 1 patient, totaling 23 patients. The mean age was 47.0 ± 12.6 years. There were 20 male and 3 female patients, respectively. The histopathological analysis of lymph nodes was conducted in 15 patients (65.2%), and the diagnosis was consistent with TAFRO syndrome in all 15 patients. Among the 23 patients, 11 patients (18 adrenal glands) showed adrenal ischemia/infarction, 9 patients (13 adrenal glands) showed adrenal hemorrhage, and 4 patients (7 adrenal glands) showed adrenomegaly without evidence of concurrent ischemia/infarction or hemorrhage. One patient demonstrated unilateral adrenal hemorrhage and contralateral adrenomegaly. In patients with adrenal ischemia/infarction, the adrenal glands displayed poor enhancement through contrast-enhanced computed tomography (CT). In patients with adrenal hemorrhage, the adrenal glands revealed high attenuation through non-enhanced CT and hematoma through magnetic resonance imaging. Adrenomegaly, with or without adrenal ischemia/infarction or hemorrhage, was observed in all patients (23/23, 100%). The subsequent calcification of the affected adrenal glands was frequently observed (9/14, 64.3%) when a follow-up CT was performed. Abdominal pain was frequent (15/23, 65.2%), all of which occurred after the disease's onset, suggesting the importance of considering TAFRO syndrome as a cause of acute abdomen. Given the absence of evidence of adrenal abnormalities in non-TAFRO-idiopathic multicentric Castleman disease (iMCD), they may serve as diagnostic clues for differentiating TAFRO syndrome from non-TAFRO-iMCD.

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.

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.

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.

A rare case of epithelial-myoepithelial carcinoma ex pleomorphic adenoma of the parotid gland: Radiologic-pathologic correlation.

Carcinoma ex pleomorphic adenoma is a carcinoma that arises from a primary or recurrent benign pleomorphic adenoma. The prevalence of epithelial-myoepithelial carcinoma is low, and this histological type accounting for only 1% of all salivary gland tumors. Here, we report a rare case of Epithelial-Myoepithelial Carcinoma ex pleomorphic adenoma of the parotid gland with a radiologic-pathologic correlation.

Performance of Neck Imaging Reporting and Data System (NI-RADS) for Diagnosis of Recurrence of Head and Neck Squamous Cell Carcinoma: A Systematic Review and Meta-analysis.

BACKGROUND: The Neck Imaging Reporting and Data System (NI-RADS) is a reporting template used in head and neck cancer posttreatment follow-up imaging. PURPOSE: Our aim was to evaluate the pooled detection rates of the recurrence of head and neck squamous cell carcinoma based on each NI-RADS category and to compare the diagnostic accuracy between NI-RADS 2 and 3 cutoffs. DATA SOURCES: The MEDLINE, Scopus, and EMBASE databases were searched. STUDY SELECTION: This systematic review identified 7 studies with a total of 694 patients (1233 lesions) that were eligible for the meta-analysis. DATA ANALYSIS: The meta-analysis of pooled recurrence detection rate estimates for each NI-RADS category and the diagnostic accuracy of recurrence with NI-RADS 3 or 2 as the cutoff was performed. DATA SYNTHESIS: The estimated recurrence rates in each category for primary lesions were 74.4% for NI-RADS 3, 29.0% for NI-RADS 2, and 4.2% for NI-RADS 1. The estimated recurrence rates in each category for cervical lymph nodes were 73.3% for NI-RADS 3, 14.3% for NI-RADS 2, and 3.5% for NI-RADS 1. The area under the curve of the summary receiver operating characteristic for recurrence detection with NI-RADS 3 as the cutoff was 0.887 and 0.983, respectively, higher than 0.869 and 0.919 for the primary sites and cervical lymph nodes, respectively, with NI-RADS 2 as the cutoff. LIMITATIONS: Given the heterogeneity of the data of the studies, the conclusions should be interpreted with caution. CONCLUSIONS: This meta-analysis revealed estimated recurrence rates for each NI-RADS category for primary lesions and cervical lymph nodes and showed that NI-RADS 3 has a high diagnostic performance for detecting recurrence.

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.

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.

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 susceptibility contrast perfusion-weighted and diffusion-weighted magnetic resonance imaging findings in pilocytic astrocytoma and H3.3 and H3.1 variant diffuse midline glioma, H3K27-altered.

OBJECTIVE: This study compared the dynamic susceptibility contrast (DSC) magnetic resonance imaging parameters and apparent diffusion coefficient (ADC) between pilocytic astrocytoma (PA) and diffuse midline glioma, H3K27-altered (DMG) variants. METHODS: The normalized relative cerebral blood volume (nrCBV), normalized relative flow (nrCBF), percentile signal recovery (PSR), and normalized mean ADC (nADCmean) of 23 patients with midline PAs (median age, 13 years [range, 1-71 years]; 13 female patients) and 40 patients with DMG (8.5 years [1-35 years]; 19 female patients), including 35 patients with H3.3- and five patients with H3.1-mutant tumors, treated between January 2016 and May 2022 were statistically compared. RESULTS: DMG had a significantly lower nADCmean (median: 1.48 vs. 1.96; p = 0.00075) and lower PSR (0.97 vs. 1.23, p = 0.13) but higher nrCBV and nrCBF (1.66 vs. 1.17, p = 0.058, respectively, and 1.87 vs. 1.19, p = 0.028, respectively) than PA. The H3.3 variant had a lower nADCmean than the H3.1 variant (1.46 vs. 1.80, p = 0.10). CONCLUSION: DMG had lower ADC and PSR and higher rCBV and rCBF than PA. The H3.3 variant had a lower ADC than the H3.1 variant. Recognizing the differences and similarities in the DSC parameters and ADC between these tumors may help presurgical diagnosis.

Dual-energy computed tomography for improved visualization of internal jugular chain neck lymph node metastasis and nodal necrosis in head and neck squamous cell carcinoma.

PURPOSE: To evaluate and compare the utility of 40-keV virtual monochromatic imaging (VMI) reconstructed from dual-energy computed tomography (DECT) in the assessment of neck lymph node metastasis with 70-keV VMI, which is reportedly equivalent to conventional 120-kVp single-energy computed tomography. MATERIALS AND METHODS: Patients with head and neck squamous cell carcinoma who had neck lymph node metastasis in contact with the sternocleidomastoid muscle (SCM) and underwent contrast-enhanced DECT were included. In 40- and 70-keV VMI, contrast differences and contrast noise ratio (CNR) between the solid component of neck lymph node metastasis (SC) and the SCM and between SC and nodal necrosis (NN) were calculated. Two board-certified radiologists independently and qualitatively evaluated the boundary discrimination between SC and SCM and the diagnostic certainty of NN. RESULTS: We evaluated 45 neck lymph node metastases. The contrast difference between SC and SCM and SC and NN were significantly higher at 40-keV VMI than at 70-keV VMI (p < 0.001). The CNR between SC and SCM was significantly higher at 40-keV VMI than at 70-keV VMI (p < 0.001). Scoring of the boundary discrimination between SC and SCM as well as the diagnostic certainty of NN at 40-keV VMI was significantly higher than that at 70-keV VMI (p < 0.001). The inter-rater agreements for these scores were higher at 40-keV VMI than at 70-keV VMI. CONCLUSION: Additional employing 40-keV VMI in routine clinical practice may be useful in the diagnosis of head and neck lymph node metastases and nodal necrosis.

MRI features of sinonasal tract angiofibroma/juvenile nasopharyngeal angiofibroma: Case series and systematic review.

BACKGROUND AND PURPOSE: To comprehensively summarize the radiological characteristics of sinonasal tract angiofibroma (STA) (commonly known as juvenile nasopharyngeal angiofibroma). METHODS: Forty-four lesions from 41 cases provided by 33 study articles identified through a systematic review and 13 lesions from 13 cases from our institution associated with patients with STA who underwent MRI were included in the review study, carried out by two board-certified experienced radiologists. RESULTS: The study participants were all male patients with a mean age of 15.6 years at the time of diagnosis. All of them presented with nasal cavity lesions (100%), predominantly in the nasopharynx (98.2%). The sphenopalatine foramen/pterygopalatine fossa was involved in 76.0%, and compressive shift of the posterolateral wall of the maxillary sinus was present in more than half (57.9%). T2-weighted imaging signal intensity was heterogeneous with mixed high and iso intensities as compared to skeletal muscle (100%). T1-weighted imaging showed partial high signal intensity in 61.1% of the cases. Flow void and intense enhancement were present in almost all cases. Cystic/nonenhancement changes on contrast-enhanced MRI were relatively common (40.8%). The mean apparent diffusion coefficient value (2.07 × 10-3  mm2 /second) and some quantitative dynamic contrast-enhanced MRI parameters were high. There was a significant difference in the frequency of residual/recurrent lesions based on the presence of MRI findings of skull base invasion (p = .017) and intracranial extension (p = .003). CONCLUSIONS: We summarized the MRI findings of STA that can facilitate timely diagnosis and appropriate management.

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.

Neuroimaging of hypophysitis: etiologies and imaging mimics.

Hypophysitis is an inflammatory disease affecting the pituitary gland. Hypophysitis can be classified into multiple types depending on the mechanisms (primary or secondary), histology (lymphocytic, granulomatous, xanthomatous, plasmacytic/IgG4 related, necrotizing, or mixed), and anatomy (adenohypophysitis, infundibulo-neurohypophysitis, or panhypophysitis). An appropriate diagnosis is vital for managing these potentially life-threatening conditions. However, physiological morphological alterations, remnants, and neoplastic and non-neoplastic lesions may masquerade as hypophysitis, both clinically and radiologically. Neuroimaging, as well as imaging findings of other sites of the body, plays a pivotal role in diagnosis. In this article, we will review the types of hypophysitis and summarize clinical and imaging features of both hypophysitis and its mimickers.

Brain MRI Findings of Hemophagocytic Lymphohistiocytosis With a Heterozygous PRF1 Gene Mutation Masquerading As CLIPPERS: A Case Report.

Familial hemophagocytic lymphohistiocytosis is a rare and potentially life-threatening genetic condition characterized by unsuppressed immune activation and hypercytokinemia. Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is a central nervous system inflammatory disorder characterized by punctate and curvilinear gadolinium-enhancing lesions in the brainstem, cerebellum, and spinal cord, which responds well to corticosteroid treatment. Hemophagocytic lymphohistiocytosis has been known to mimic CLIPPERS on neuroimaging, and patients previously diagnosed with CLIPPERS may carry familial hemophagocytic lymphohistiocytosis-related gene mutations that serve as predisposing factors. In this article, we describe a case initially diagnosed with CLIPPERS based on characteristic magnetic resonance imaging features and clinical course, who was later diagnosed with hemophagocytic lymphohistiocytosis based on a heterozygous familial hemophagocytic lymphohistiocytosis-associated PRF1 gene mutation.

Advanced imaging of head and neck infections.

When head and neck infection is suspected, appropriate imaging contributes to treatment decisions and prognosis. While contrast-enhanced CT is the standard imaging modality for evaluating head and neck infections, MRI can better characterize the skull base, intracranial involvement, and osteomyelitis, implying that these are complementary techniques for a comprehensive assessment. Both CT and MRI are useful in the evaluation of abscesses and thrombophlebitis, while MRI is especially useful in the evaluation of intracranial inflammatory spread/abscess formation, differentiation of abscess from other conditions, evaluation of the presence and activity of inflammation and osteomyelitis, evaluation of mastoid extension in middle ear cholesteatoma, and evaluation of facial neuritis and labyrinthitis. Apparent diffusion coefficient derived from diffusion-weighted imaging is useful for differential diagnosis and treatment response of head and neck infections in various anatomical sites. Dynamic contrast-enhanced MRI perfusion may be useful in assessing the activity of skull base osteomyelitis. MR bone imaging may be of additional value in evaluating bony structures of the skull base and jaw. Dual-energy CT is helpful in reducing metal artifacts, evaluating deep neck abscess, and detecting salivary stones. Subtraction CT techniques are used to detect progressive bone-destructive changes and to reduce dental amalgam artifacts. This article provides a region-based approach to the imaging evaluation of head and neck infections, using both conventional and advanced imaging techniques.

Neuroimaging features of FOXR2-activated CNS neuroblastoma: A case series and systematic review.

BACKGROUND AND PURPOSE: CNS neuroblastoma, FOXR2-activated (CNS NB-FOXR2) is a newly recognized tumor type in the 2021 World Health Organization classification of central nervous system (CNS) tumors. We aimed to investigate the clinical and neuroimaging findings of CNS NB-FOXR2 and systematically review previous publications and three new cases. METHODS: We searched PubMed, SCOPUS, and Embase databases for patients with pathologically proven CNS NB-FOXR2 with sufficient information for preoperative CT and MRI findings. Two board-certified radiologists reviewed the studies and imaging data. RESULTS: Thirty-one patients from six previous publications and 3 patients from our hospital comprised the study population (median age, 4.2 [range: 1.4-16] years; 19 girls). Clinically, CNS NB-FOXR2 mainly affected children between 2 and 6 years (24/34, 67.6%). Nausea/vomiting and seizures were reported as the main presenting symptoms (100% in total). The tumors frequently showed hyperdensity compared to the cortex on nonenhanced CT (4/5, 80%) with calcification along the inner rim of the tumor (4/5, 80%). More than half of patients showed susceptibility artifacts indicating intratumoral hemorrhage and/or calcification (15/28, 53.6%) on T2*- and/or susceptibility-weighted imaging. Elevated relative cerebral blood volume and flow and percentile signal recovery were observed in one case with dynamic susceptibility contrast MRI. CONCLUSIONS: Characteristic imaging features including hyperdense attenuation of the solid components and calcification along the inner rim on CT and susceptibility-weighted imaging may assist with preoperative diagnosis of CNS NB-FOXR2 in pediatric patients.