Ring-like Enhancement Research Articles

Abstract 4136394: Elucidation of a novel genetic substrate responsible for genetically elusive ring-like arrhythmogenic cardiomyopathy

Background: Arrhythmogenic cardiomyopathy (ACM) is a genetic heart disease characterized by fibrofatty replacement of ventricular myocardium. Arrhythmogenic left ventricular (LV) cardiomyopathy (ALVC) is a phenotypic variant of ACM predominantly involving the LV. Mutations in genes encoding for desmosome proteins and filamin C have been implicated in ALVC, especially in the setting of a ring-like late gadolinium enhancement (LGE) imaging pattern. Objective: To identify the underlying genetic substrate responsible for genetically elusive ring-like ALVC in a patient with seemingly sporadic/recessive disease. Methods: A 38-year-old male was referred for genetic investigation to determine the underlying genetic substrate responsible for his ALVC phenotype. The patient fulfilled both electrocardiographic (low voltage in the limb leads and anterolateral T-wave inversions) and imaging (>1 segment of subepicardial LGE) criteria for ALVC. Additionally, the patient experienced unexplained episodes of tachycardia, shakiness, sweating, and nausea prompting concern for an underlying neuromuscular disease. Genome sequencing (GS) was performed on DNA isolated from the affected patient and both of his unaffected parents. Variants were filtered assuming either a sporadic de novo occurrence or an autosomal recessive inheritance pattern. Results: GS identified compound heterozygous GNPTAB variants in the affected patient. A previously reported maternally derived p.Leu257Leu (c.771G>A) variant involving the last nucleotide of exon 7 of the GNPTAB gene and a novel paternally inherited Lys834fs*5 frame-shift variant were identified. The p.Leu257Leu variant has been reported to cause aberrant splicing and exon skipping of the GNPTAB mRNA transcript. Pathogenic variants in the GNPTAB encoded N-acetylglucosamine-1 (GlcNAc)-phosphotransferase subunits alpha/beta cause mucolipidosis type III, a rare recessive lysosomal storage disease characterized by coarse facial features, developmental delay, short stature, skeletal deformities, and cardiac involvement including valvular thickening and cardiomyopathy. Conclusions: Here, using a genome sequencing trio analysis we identified a compound heterozygous GNPTAB pathogenic variants as the underlying genetic substrate in a patient with ring-like ALVC and concomitant neuromuscular disease manifestation. Genome sequencing may identify the genetic etiology responsible for an otherwise undifferentiated diagnosis of cardiomyopathy.

Ringlike late gadolinium enhancement: prevalence, association with cardiac phenotype, and patient outcome in an unselected cohort referred for cardiovascular magnetic resonance imaging

Abstract Background The identification of a non-ischemic ringlike enhancement in the left ventricular (LV) myocardium by cardiac magnetic resonance imaging (CMR) has emerged as a significant biomarker associated with arrhythmogenic cardiomyopathy (CMP) and adverse patient outcomes. Recently, the term "scarring CMP" has been also proposed to encompass various conditions characterized by a high burden of non-ischemic, often ring-like, myocardial scarring, including both genetic and acquired etiologies. Purpose To systematically evaluate CMR phenotypic traits and etiologic backgrounds in patients with ringlike enhancement and to assess the burden of adverse outcomes during follow-up (FU). Methods This is a single-center, retrospective analysis of prospectively enrolled patients undergoing CMR (from 2002 to 2024). Ringlike late gadolinium enhancement (LGE) was defined as continuous enhancement in three or more adjacent segments based on the AHA 17 segment model. Patients records were reviewed for etiologic classification and FU assessment. Results Among 23.472 patients, 19.696 (84%) underwent LGE assessment. In 152 patients (0.77%; 73% male; mean age 48.5 ± 17 years) a ringlike LGE was identified. The mean number of LV segments with LGE was 10 ± 3. Right ventricular (RV) LGE was present in 23% of subjects. LV fatty metaplasia was detected in 22%. Infero-lateral segments were most frequently affected. CMR showed the following morpho-functional features: LV dilatation (53%), LV hypokinesia (69%), LV hypertrophy (10.5%), LV non-compaction (11%), RV dilation (26%), and RV ejection fraction < 40% (21%). Phenotypic classification according to 2023 ESC guidelines identified dilated CMP phenotype (43%), non-dilated LV CMP phenotype (39%), predominant arrhythmogenic right ventricular phenotype (6%), hypertrophic phenotype (7%). 5% of cases remained unclassified, all of them showing LV dilated non-hypokinetic phenotype. Genetic analysis was performed in 101 patients (66%). Etiologic classification revealed non-desmosomal gene related CMP (24%), desmosomal gene related CMP (8%), inflammatory CMP (16%), genetic neuromuscular diseases (5%), congenital metabolic errors (3%), familiar gene elusive CMP (2%) and unknown etiology (45%). Notably, only 17% of patients classified as having inflammatory CMP and 66% of those with unknown etiology underwent genetic testing, with negative results. The median follow-up duration was 3 (IQR: 1-7) years. All-cause mortality occurred in 10.5% of patients, while 17% experienced sustained ventricular tachycardia, appropriate implantable cardioverter-defibrillator therapies, or sudden cardiac death. Heart transplantation or left ventricular assist device implantation was required in 8% of cases. Conclusion Ringlike LGE is a rare, non-disease-specific feature that can be found in different morpho-functional CMP phenotypes. It is associated with frequent genetic-determined etiology and high burden of arrhythmic and non-arrhythmic outcomes.

Role of gadoxetic acid-enhanced MRI in the differential diagnosis of chemotherapy-induced focal nodular hyperplasia-like lesions and liver metastases in patients with colorectal cancer.

To describe the gadoxetic acid-enhanced magnetic resonance imaging (MRI) features and follow-up changes of hepatic focal nodular hyperplasia (FNH)-like lesions induced by chemotherapy in patients with colorectal cancer (CRC) and the differential diagnosis of FNH-like lesions and liver metastases. We retrospectively analyzed the data of patients with CRC who received chemotherapy and gadoxetic-enhanced MRI at our hospital. Based on imaging features and pathological findings, the patients were classified into two groups: FNH-like lesions and liver metastases. Two abdominal radiologists reviewed and compared the signal intensities of all images in each phase for both groups. The characteristics of the FNH-like lesions in the hepatobiliary phase were evaluated, and changes in size of lesions were monitored. Thirty patients with 82 FNH-like lesions and 30 with 49 liver metastases following chemotherapy were included in the study. All MRI findings were statistically significantly different between the two groups (p < 0.05). In FNH-like lesions, three enhancement patterns were observed in the hepatobiliary phase: hyperintense/isointense (18.3%), heterogeneous hyperintense (8.5%), and ring-like enhancement (73.2%). The median time from completion of chemotherapy to development of FNH-like lesions was 31 months. During 4-87 months of follow-up, 27 patients with 73 lesions showed the following outcomes: 41 lesions (56.16%) showed stability, 21 lesions (28.77%) growth, and 11 lesions (15.07%) reduction or disappearance. Gadoxetic acid-enhanced MRI can distinguish between chemotherapy-induced FNH-like lesions and liver metastases in patients with CRC. The FNH-like lesions exhibited three enhancement patterns in the hepatobiliary phase, and the changes varied during follow-up.

Prognostic significance of MRI contrast enhancement in newly diagnosed glioblastoma, IDH-wildtype according to WHO 2021 classification.

Contrast enhancement in glioblastoma, IDH-wildtype is common but not systematic. In the era of the WHO 2021 Classification of CNS Tumors, the prognostic impact of a contrast enhancement and the pattern of contrast enhancement is not clearly elucidated. We performed an observational, retrospective, single-centre cohort study at a tertiary neurosurgical oncology centre (January 2006 - December 2022). We screened adult patients with a newly-diagnosed glioblastoma, IDH-wildtype in order to assess the prognosis role of the contrast enhancement and the pattern of contrast enhancement. We included 1149 glioblastomas, IDH-wildtype: 26 (2.3%) had a no contrast enhancement, 45 (4.0%) had a faint and patchy contrast enhancement, 118 (10.5%) had a nodular contrast enhancement, and 960 (85.5%) had a ring-like contrast enhancement. Overall survival was longer in non-contrast enhanced glioblastomas (26.7 months) than in contrast enhanced glioblastomas (10.9 months) (p < 0.001). In contrast enhanced glioblastomas, a ring-like pattern was associated with shorter overall survival than in faint and patchy and nodular patterns (10.0 months versus 13.0 months, respectively) (p = 0.033). Whatever the presence of a contrast enhancement and the pattern of contrast enhancement, surgical resection was an independent predictor of longer overall survival, while age ≥ 70 years, preoperative KPS score < 70, tumour volume ≥ 30cm3, and postoperative residual contrast enhancement were independent predictors of shorter overall survival. A contrast enhancement is present in the majority (97.7%) of glioblastomas, IDH-wildtype and, regardless of the pattern, is associated with a shorter overall survival. The ring-like pattern of contrast enhancement is typical in glioblastomas, IDH-wildtype (85.5%) and remains an independent predictor of shorter overall survival compared to other patterns (faint and patchy and nodular).

MRI features of ovarian teratomas with somatic-type malignancy and mature cystic teratomas.

We evaluated the magnetic resonance imaging (MRI) features of ovarian teratomas with somatic-type malignancy (TSMs) and benign ovarian mature cystic teratomas (MCTs) to determine the diagnostic contribution of the MRI findings for differentiating these two teratomas. We compared the MRI findings between ovarian TSMs (n = 10) and MCTs (n = 193), and we conducted a receiver operating characteristic (ROC) analysis to determine the MRI findings' contribution to the differentiation of TSMs from MCTs. The maximum diameters of whole lesion and the largest solid component in the TSMs were larger than those of the MCTs (p = 0.0001 and p < 0.0001, respectively). Fat tissue in solid components was seen in 73/116 (62.9%) MCTs but in none of the TSMs (p = 0.0001). Ring-like enhancement in solid components was seen in 60/116 (51.7%) MCTs and none of the TSMs (p = 0.0031). On dynamic contrast-enhanced MRI (DCE MRI), all of the solid components in the TSMs showed a high- or intermediate-risk time intensity curve (TIC), and those in 113 of the 116 (97.4%) MCTs showed a low-risk TIC (p < 0.0001). The area under the curve of the ROC analysis using the high-/intermediate-risk TIC on DCE MRI was the highest (0.99) for differentiating TSMs from MCTs: sensitivity 100%, specificity 97.4%, positive predictive value 75.0%, negative predictive value 100%, and accuracy, 97.6%. Compared to ovarian MCTs, ovarian TSMs are larger and have larger solid components with high- or intermediate-risk TICs on DCE MRI. Ovarian MCTs frequently show small solid components with fat tissue, ring-like enhancement, and a low-risk TIC on DCE MRI.

Triple-Negative or Close-to-Triple-Negative Breast Cancer Presenting as a Thick-Walled Cystic Lesion.

Clinical and pathologic characteristics of the invasive ductal carcinoma (IDC) presenting as a thick-walled breast cyst are little known. Three female patients were included in this report. A palpable, nontender breast lump was found in all cases. While mammography showed a hyperdense mass, ultrasonography demonstrated a thick-walled cystic mass. Magnetic resonance imaging clearly showed the cystic breast lesions with ring-like or irregular rim enhancement. A grade III IDC was confirmed in all cases. All IDCs but one were estrogen receptor negative, progesterone receptor negative, and human epidermal growth factor receptor 2 negative, with merely weak progesterone receptor positivity (5%) in one case. All cases underwent surgical management first and postoperative chemotherapy. Breast malignancy presenting as a thick-walled cystic mass could be a highly aggressive IDC, even triple-negative breast cancer. It is imperative for breast cancer-related practitioners to identify the potentially malignant cystic lesions timely and adopt appropriate management.

Development and Validation of a Nomogram Based on Multimodality Ultrasonography Images for Differentiating Malignant from Benign American College of Radiology Thyroid Imaging, Reporting and Data System (TI-RADS) 3–5 Thyroid Nodules

ObjectivesTo develop and validate a predictive nomogram based on combined image features of gray-scale ultrasonography (US), elastosonography (ES), and contrast-enhanced US (CEUS) to differentiate malignant from benign ACR TI-RADS3-5 thyroid nodules. Materials and MethodsAmong 2767 thyroid nodules scanned by CEUS in * Hospital between April 2014 and November 2018, 669 nodules classified as ACR TI-RADS 3-5 were included with confirmed diagnosis and ES examination. 455 nodules were set as a training cohort and 214 as a validation cohort. Images were categorized as gray-scale US ACR TI-RADS3, TI-RADS4, TI-RADS5; ES patterns of ES-1, ES-2; and CEUS patterns of either heterogeneous hypoenhancement, concentric hypoenhancement, homogeneous hyper/isoenhancement, no-perfusion, hypoenhancement with sharp margin, island-like enhancement, or ring-like enhancement. Based on multivariate logistic regression analysis, a predictive nomogram model was developed and validated by receiver operating characteristic curve analysis. ResultsIn the training cohort, ACR TI-RADS4-5, ES-2, heterogeneous hypoenhancement, concentric hypoenhancement, and homogeneous hyper/isoenhancement were selected as predictors of malignancy by univariate logistic regression analysis. A predictive nomogram (combining indices of ACR TI-RADS, ES and CEUS) showed excellent predictive ability for differentiating malignant from benign lesions in the training cohort (AUC, 0.93 [95% CI 0.90-0.95]). The prediction nomogram model demonstrated a sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of 0.84, 0.88, 0.91 and 0.81.In the validation cohort, the AUC of the prediction nomogram model was significantly higher than those of the single modalities (P<0.005) . The AUCs of the validation cohort were 0.93 (95% CI 0.89-0.96) and 0.93 (95% CI 0.89-0.97) respectively for senior and junior radiologists. The prediction nomogram model has a sensitivity, specificity, PPV and NPV of 0.86, 0.87, 0.87 and 0.86. ConclusionsA predictive nomogram model combining ACR TI-RADS, ES, and CEUS showed potential clinical utility in differentiating malignant from benign ACR TI-RADS3-5 thyroid nodules.

10249-PEDT-8 CURRENT STATUS AND ISSUES OF PEDIATRIC DIFFUSE INTRINSIC PONTINE GLIOMA (DIPG)

Abstract that predominantly occurs in childhood, with a median survival of less than one year. Herein we present a recent case of DIPG and discuss the current status and future challenges. The patient was a 7-year-old boy who complained of tinnitus, headaches, and unstable gait. The MRI showed diffuse enlargement of the pons, and the contrast-enhanced T1-weighted image exhibited an irregular ring-like enhancement. Neurologically, he presented with left abducens nerve palsy, horizontal gaze nystagmus, significant left ataxia, and truncal ataxia. As the lesion extended to the left cerebellar peduncle, a stereotactic biopsy was performed. However, postoperative bleeding occurred, resulting in transient consciousness disturbance, left facial palsy, speech disturbance, and dysphagia, necessitating enteral nutrition management. The pathological diagnosis was high-grade glioma, IDH wild type, H3K27M mutation (-), TERTp wild type, BRAF V600E mutation (-), BRAF fusion (-), and MGMT unmethylated. After surgery, the patient received radiation therapy (54Gy/30fr), and his symptoms improved, allowing him to resume oral intake. Post-irradiation, the lesion shrank, and he was discharged home without assistance. However, six months later, his symptoms worsened, and he started taking temozolomide orally, but the effect was limited. Nine months later, he became unable to take oral medication, and a central venous port was placed. We coordinated with home care physicians to provide intravenous and palliative treatments, but respiratory and consciousness disturbances gradually worsened, and he died 10 months after the initial diagnosis. The median survival of DIPG patients treated at our hospital is currently 10.5 months, and this case followed a typical course. The development of novel treatments is desired, but due to the rarity of the disease, conducting comparative trials is challenging. Hence, registry data forming a control group are necessary. Additionally, transitioning to home-based palliative care and sharing information on supportive therapies are also critical tasks.

JS04.6.A CLINICAL PRESENTATION OF “MOLECULAR GLIOBLASTOMA” IS DIFFERENT COMPARED TO “HISTOLOGICAL GLIOBLASTOMA”, BUT OUTCOME IS THE SAME, REGARDLESS OF GRADE OR PRESENCE OF ONLY ATERT PROMOTER MUTATION

Abstract BACKGROUND In the 2021 revised WHO CNS, IDH1/2-wildtype (IDHwt) gliomas with histological features of lower grade glioma, but with a TERT-promotor (TERTp) mutation, and/or EGFR amplification, and/or combined gain of chromosome 7 and loss of chromosome 10 (7+/10-), are now classified as IDHwt glioblastoma. These formerly labeled “molecular glioblastomas” (m-GBMs) are not separated from histologically defined glioblastomas (h-GBMs). However, there is still discussion whether these gliomas are truly the same in terms of first presentation and survival especially in the presence of grade 2 histology. We expanded and re-analyzed a cohort of m-GBMs and had the histological grade re-evaluated, to investigate the impact of histological grade in m-GBMs, and also specifically in patients with isolated TERTp mutations. MATERIAL AND METHODS We identified cases from 3 Dutch hospitals, which were initially histologically classified as grade 2 or 3 glioma, but molecularly as m-GBM. Cases with ring-like contrast enhancement and/or central necrosis at time of diagnostic surgery were excluded. Two dedicated neuropathologists blinded for clinical information independently revised the histological grade as defined in previous WHO classifications. Discordant cases were discussed to reach consensus. Patients were classified with a tailored NGS panel. We compared overall survival (OS) in this cohort with OS of a historical h-GBM cohort. RESULTS We identified 102 cases. Out of the 81 grade 2 gliomas (original diagnostics), upon pathology review 60 patients were still classified as grade 2 tumors, in 15 patients the grade was revised into grade 3, and in 6 patients into grade 4. Out of the 21 grade 3 patients, 11 were again classified as grade 3 after revisions. Five patients were revised as grade 2, and the other 5 as grade 4. Median age did not differ between groups (grade 2: 61yrs, grade 3: 58yrs, grade 4: 64yrs, h-GBM: 55 yrs). Clinical presentation of m-GBM was different compared to h-GBM. m-GBM more often presented with seizures than h-GBM (66% vs 27%) and more often with gliomatosis cerebri (35% vs 17%) reflected by a higher percentage of biopsies (78% vs 17%). We found no significant OS difference between histological grades in m-GBM and no difference with the historical h-GBM cohort. Median OS for grade 2 patients was 17 months versus 18 months in h-GBM patients. Additionally, we found no difference in OS between patients with an isolated TERTp mutation versus with EGFR amplification and/or 7+/10- in histological grade 2 patients (Median OS of 17 months versus 21 months respectively). CONCLUSION In our cohort of m-GBM with a low-grade histology and imaging characteristics of low-grade glioma, we observed a different clinical presentation compared to h-GBM. We did not see an impact of histological grade on OS in the context of m-GBM, and we did not see a longer survival for patients with an isolated TERTp mutation.

Pseudocystic inflammatory demyelinating lesions in multiple sclerosis: A clinical, radiological, and pathological description.

Pseudocystic inflammatory demyelinating lesions (PIDLs) are poorly described in MS and might represent a diagnostic challenge. We described the clinical, radiological, pathological, and follow-up characteristics of 13 PIDL in 9 MS patients. We constituted a single-center retrospective case series of PIDLs in MS, defined on MRI as expansive cyst-like lesions, with a fluid-signal content, and a diameter of 1 cm or more. PIDL often occurred at first event (56%), were often asymptomatic (69%), and encircled by a hypo-T2 diffusion-restricted rim and a thin ring-like gadolinium enhancement (100%) on magnetic resonance imaging (MRI). Associated typical MS lesions were constant. Biopsies from two PIDLs displayed classical features of active MS, except for unusual edema. PIDLs are clinically unremarkable and associated with a good outcome. Their easily recognizable MRI features could help avoid biopsy.

A Nomogram Based on Contrast-Enhanced Ultrasound to Predict the Microvascular Invasion in Hepatocellular Carcinoma

The aim of this study was to establish and validate a contrast-enhanced ultrasound (CEUS) nomogram for pre-operative microvascular invasion (MVI) prediction in hepatocellular carcinoma (HCC), and compare it with the nomogram based on gadopentetate dimeglumine-enhanced magnetic resonance imaging (Gd-MRI). A total of 251 patients with a single HCC were enrolled in this prospective study, including 176 patients in the training cohort and 75 patients in the validation cohort. Contrast-enhanced ultrasound (CEUS) with Sonazoid and Gd-MRI was performed pre-operatively. Post-operative histopathology was the gold standard for MVI. Univariate and multivariate logistic regression was performed to determine independent risk factors for MVI. Nomograms based on CEUS and Gd-MRI were established, and their discrimination, calibration and decision curve analysis were evaluated and compared. Multivariate logistic regression revealed that arterial circular enhancement, non-enhancing area and thick ring-like enhancement in the post-vascular phase were independent risk factors for MVI. The areas under the receiver operating characteristic curve of the nomogram were 0.841 (0.779-0.892) and 0.914 (0.827-0.966) in the training and validation cohorts, with no significant difference compared with the Gd-MRI nomogram (p=0.294, 0.321). The C-indexes were 0.821 and 0.870 in the training and validation cohorts. Decision curve analysis revealed that the CEUS nomogram had better clinical applicability than the Gd-MRI nomogram when the threshold probability was between 0.35 and 0.95. The CEUS-based nomogram was available for predicting MVI in HCC, and its predictive performance was not inferior to that of Gd-MRI.

Intramedullary Schwannoma of the Spinal Cord: A Nationwide Analysis by the Neurospinal Society of Japan.

This study was aimed to report the clinical characteristics of intramedullary schwannomas and discuss imaging findings and treatment strategies. The inclusion criterion was consecutive patients with intramedullary schwannomas who were surgically treated at eight centers between 2009 and 2020. Clinical characteristics included age, sex, clinical presentation, disease duration, and follow-up period. The modified McCormick scale was used to compare the preoperative and postoperative conditions. Pre- and postoperative magnetic resonance images (MRI) of each case were analyzed. The mean age of the total 11 patients at the operation was 50.2 years. The mean duration of the symptoms was 23 months, with limb paresthesia being the most common clinical presentation. The cervical spine was the most common localization level of the tumor in 6 cases. The mean follow-up duration was 49.4 months. Gross total resection (GTR) and subtotal resection (STR) was achieved in 9 and 2 cases, respectively. According to the modified McCormick scale at 6 months postoperatively, 7 cases (63.6%) had improved and 4 cases (36.3%) had unchanged grades. Typical MRI findings of the intramedullary schwannoma included ring-like enhancement, syringomyelia, cystic formation, intramedullary edema, and hemosiderin deposition. Gadolinium enhancement was homogenous in 8 cases (72.7%). The tumor margins were well demarcated in all cases. Intramedullary schwannoma should be considered when sharp margins and well-enhanced tumors are present at the cervical spine level and the initial symptoms are relatively mild, such as dysesthesia. When GTR cannot be achieved, STR for tumor decompression is recommended.

RADIATION NECROSIS AFTER CAVERNOMA RADIOSURGERY SIMULATING BRAIN HIGH-GRADE GLIOMA – A CASE REPORT AND REVIEW OF THE LITERATURE

We report a 62-year-old female patient who came to our institution due to pain and limited movements in the neck area, nausea, and headache. The magnetic resonance imaging (MRI) revealed a single larger and two smaller lesions with the appearance of cavernomas in the left parietal brain region. The patient was consulted with a neurosurgeon and was judged to be inoperable. Radiosurgery was performed in ​​the cavernoma with a total focal dose of 15Gy. 11 months after the radiotherapy, during a routine follow-up MRI scan, a tumor-like lesion with extensive perifocal edema and midline dislocation, with heterogenous structure and ring-like enhancement was reported in the left parietal brain region. After follow-up for 15 months, the last MRI showed almost complete reverse development of the described lesion, with marked reduction of volume, edema, and contrast enhancement. Along with MRI features of the lesion, some of which overlap with high-grade glioma characteristics, the evolution of the lesion is in favor of a radiation necrosis zone.

DISTRIBUTION AND IMAGING CHARACTERISTICS OF HIGH-GRADE GLIOMAS ACCORDING TO METHYLATION STATUS OF MGMT GENE PROMOTER

Background: Many molecular investigations have identified that the methylation status of the O-6-methylguanine-DNA methyl transferase (MGMT) gene promoter in patients with high grade glioma is associated with a improved prognosis and survival when treated with temozolamide (TMZ). Aims and Objective: The aim of the study was to find out the distribution of methylation status and imaging characteristics in patients with High Grade Gliomas. Method: The study included forty consecutive patients who presented with neurological symptoms and were subsequently diagnosed as having a primary brain tumour on conventional MRI scan. MR-perfusion imaging was performed to determine the grade of the tumor which was subsequently compared with histopathological grade of the tumor after brain biopsy. The biopsy or surgical specimens were further analysed for determining the MGMT promoter methylation status using Methylation-specific PCR (MSP). Results: The mean age of patients was 50.3 years in the methylated group and 49.1 years in the unmethylated group (p value = 0.671). Out of 40 patients, 18 had methylated MGMT promoters while 22 had unmethylated MGMT promoters. We found that ring-like enhancement was seen more frequently in the unmethylated tumours (72.7%) than in methylated ones (61.1%). The presence of T2 heterogeneous signal intensity was seen more in the methylated group (77.8%) than unmethylated group (68.2%). Severe necrosis (affecting &gt;50% of the tumour) was seen more in the methylated tumours (61.1%) than in the unmethylated tumours (40.09%). However, these differences were found to be statistically insignificant. Conclusion: The distribution and imaging characteristics may provide some information about methylation of high-grade gliomas but a multicentric large data maybe required to reach a consensus about its reliability when biopsy of the methylation status of the tumour is not available.

Ring-like enhancement on Contrast-Enhanced ultrasound may correlate well with histopathological capsule of Hepatocellular Carcinoma: Comparison with the enhancing capsule on Gadopentetate-enhanced MRI

PurposeTo evaluate the correlation of ring-like enhancement (RE) on contrast-enhanced ultrasound with Sonazoid (S-CEUS) with the fibrous capsule of HCC, and compared it with the enhancing capsule on Gadopentetate-enhanced MRI (Gd-MRI). MethodConsecutive patients with pathologically-proven resected HCCs from January 2021 to December 2021 were reviewed in this retrospective study. Preoperatively, the RE on the S-CEUS and the enhancing capsule on Gd-MRI were evaluated for each lesion. We analyzed the correlation of the presence, integrity, and thickness of imaging features with the histopathological fibrous capsule. The diagnostic performance to recognize the capsule between two modalities was compared by the McNemar test. ResultsNinety-one patients (mean age, 58.30 ± 11.13 years; 82 men) with ninety-seven HCCs (mean size, 4.67 ± 2.70 cm) were reviewed. The RE on S-CEUS correlated with the presence of the fibrous capsule (P = 0.006). The sensitivity, specificity, and accuracy were 92.05 %, 44.44 %, and 82.47 %, respectively, which was not different from Gd-MRI (P = 0.388). The integrity of RE in the post-vascular phase correlated with the integrity of the fibrous capsule (P = 0.018), whose diagnostic performance was not statistically different from Gd-MRI (P > 0.999). The thickness shown both on S-CEUS and Gd-MRI was thicker than that in histopathology (P < 0.001), and the thickness on Gd-MRI was not statistically different from that in the vascular phase of S-CEUS (P = 0.563), but thinner than that in the post-vascular phase (P = 0.001). ConclusionsCompared with the Gd-MRI, RE on S-CEUS had a similarly well correlation with the presence and integrity of fibrous capsule of HCC.

Prognosis evaluation of chronic inflammatory cardiomyopathy with ring-like late gadolinium enhancement.

Ring-like late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) imaging is a special LGE pattern in patients with chronic inflammatory cardiomyopathy (infl-CMP), which is associated with cardiac dysfunction and ventricular remodelling and attributed to viral infection followed by bacterial and parasitic infections. Data on the combination of CMR imaging and clinical parameters that can add long-term prognostic value in patients with infl-CMP are still rare. We aimed to evaluate the prognostic value of CMR in risk stratification in this kind of patients. A total of 319 consecutive patients with clinically suspected myocarditis were retrospectively identified. Forty-seven patients with ring-like LGE on CMR who diagnosed as infl-CMP, and 72 patients with other LGE pattern were eligible for standardized follow-up. The left ventricle (LV) and right ventricle function and mass were analyses by CMR. Myocardial strain of the ventricles was evaluated by feature tracking. Major (cardiac death, resuscitated cardiac arrest, ventricular assist device, transplantation, and appropriate implantable cardioverter-defibrillator shock) and minor (rehospitalization due to heart failure and sustained atrial fibrillation) adverse cardiovascular events were assessed during follow-up since the date of nCMR examination. Cox proportional hazards model was used to investigate which of the prognostic factors identified by univariable analysis were significantly associated with cardiac events. In the ring-like LGE group, adverse cardiac events occurred in 14 (31.11%) patients, including 7 deaths (15.56%), 6 (13.33%) heart-failure hospitalizations, and 1 (2.22%) case of sustained atrial fibrillation during the mean follow-up period of 70.15±45.68months (interquartile range: 32.83-103.71months). No major or minor adverse cardiac event occurred in the other LGE pattern group, except rehospitalization in one patient due to arrhythmia. Further analyses of ring-like LGE group by univariable and Multivariable Cox proportional hazard regression analysis showed that body mass index (BMI) (hazard ratio [HR]: 0.54, 95% confidence interval [CI]: 0.40-0.73, P=0.000), right ventricle cardiac index (RVCI) (HR: 0.31, 95% CI: 0.14-0.71, P=0.002), LV basal peak circumferential strain (LV-PCSbasal ) (HR: 1.26, 95% CI: 1.11-1.43, P=0.000) were independently associated with the long-term outcome. Receiver operator characteristic curve indicated that the cut-off of LV-PCSBasal was -7.95%, and it has added prognostic value to BMI and RVCI. For infl-CMP patients with ring-like LGE on CMR, low BMI and RVCI were associated with a poor prognosis. LV-PCSBasal with a cut-off of -7.95% can add prognostic value for patients with infl-CMP who have ring-like LGE.

Brain abscess in a patient with generalized dystonia after deep brain stimulation: illustrative case.

Infections related to deep brain stimulation (DBS) devices are not rare, but abscess formation in brain parenchyma is extremely rare. A 50-year-old man with generalized dystonia had undergone DBS of bilateral globus pallidus internus. The authors attempted to remove the bilateral DBS system due to repeated device infections caused by metal allergies. However, the intracranial lead had to be left in place, because the lead was strongly adherent to brain parenchyma. Five years later, magnetic resonance imaging showed ring-like enhancement localized around the tip of the intracranial lead, suggesting brain abscess. In response to the symptoms, the remaining left intracranial electrode was removed. Brain abscesses require several months of treatment with appropriate antibiotics, but good outcomes can be achieved with appropriate treatment. Brain abscess is a rare complication of DBS. In the present case, the infection spread from the subcutaneous infected foci to the intracranial area through the lead, resulting in the formation of a brain abscess. Removing as much of the device as possible from the body is therefore important, even if adhesions with brain parenchyma or other tissues are present, because of the risk of serious complications, as seen in this case.

Ringlike late gadolinium enhancement provides incremental prognostic value in non-classical arrhythmogenic cardiomyopathy

BackgroundThe 2019 arrhythmogenic right ventricular cardiomyopathy (ARVC) risk model has proved insufficient in the capability of predicting ventricular arrhythmia (VA) risk in non-classical arrhythmogenic cardiomyopathy (ACM). Furthermore, the prognostic value of ringlike late gadolinium enhancement (LGE) of the left ventricle in non-classical ACM remains unknown. We aimed to assess the incremental value of ringlike LGE over the 2019 ARVC risk model in predicting sustained VA in patients with non-classical ACM. MethodsIn this retrospective study, consecutive patients with non-classical ACM who underwent CMR from January 2011 to January 2022 were included. The pattern of LGE was categorized as no, non-ringlike, and ringlike LGE. The primary outcome was defined as the occurrence of sustained VA. Univariable and multivariable Cox regression analysis was used to evaluate the impact of LGE patterns on sustained VA and area under curve (AUC) was calculated for the incremental value of ringlike LGE. ResultsA total of 73 patients were collected in the final cohort (mean age, 39.3 ± 14.4 years, 51 male), of whom 10 (13.7%) had no LGE, 33 (45.2%) had non-ringlike LGE, and 30 (41.1%) had ringlike LGE. There was no statistically significant difference in the 5-year risk score among the three groups (P = 0.190). During a median follow-up of 34 (13–56) months, 34 (46.6%) patients experienced sustained VA, including 1 (10.0%), 13 (39.4%) and 20 (66.7%) of patients with no, non-ringlike and ringlike LGE, respectively. After multivariable adjustment, ringlike LGE remained independently associated with the presence of sustained VA (adjusted hazard ratio: 6.91, 95% confidence intervals: 1.89–54.60; P = 0.036). Adding ringlike LGE to the 2019 ARVC risk model showed significantly incremental prognostic value for sustained VA (AUC: 0.80 vs. 0.67; P = 0.024). ConclusionRinglike LGE provides independent and incremental prognostic value over the 2019 ARVC risk model in patients with non-classical ACM.

18F-FDG PET/CT and MR imaging features of liver metastases in gastrointestinal stromal tumors: a cross-sectional analysis

Early detection of gastrointestinal stromal tumor (GIST) liver metastases is crucial for the management and prognosis. In our experience, GIST liver metastases can display hypermetabolism on 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) and marked enhancement on magnetic resonance imaging (MRI), which are uncommon in other tumors before treatment. Most literature focus on the imaging evaluation, prognosis after treatment and less is known about imaging features on both imaging methods before treatment. This study analyzes the imaging features of newly diagnosed GIST liver metastases on 18F-FDG PET/CT and MRI, with goal of improving diagnostic accuracy. This retrospective study included 55 patients with pathological or radiographical confirmed GIST liver metastases who underwent PET/CT (n=29), MRI (n=22), or both methods (n=4). PET/CT and MRI interpretation including lesion's morphologic features, number, density or signal intensity, hemorrhage, cystic changes or necrosis, maximum standardized uptake value (SUVmax) of liver metastases and liver background on PET imaging, degree and pattern of enhancement on MRI were obtained by two experienced nuclear medicine physicians and two radiologists respectively. Data are presented as numbers, percentages, means ± standard deviations or median (interquartile range). The correlation between diameter and SUVmax of metastases, and primary tumor SUVmax and synchronous liver metastases SUVmax were analyzed by Spearman's rank test. On PET/CT visual analysis, 38.9%, 23.9%, and 37.2% of lesions showed significant hypermetabolism, slightly higher metabolism, and equal or lower metabolism than liver, respectively. There was a weak correlation between the diameter and SUVmax of liver metastases (rs =0.370, P<0.001), and a moderate correlation between SUVmax of synchronous liver metastases and the primary tumors (rs =0.492, P<0.001). On contrast-enhanced MRI, 90.8% of lesions showed heterogeneous enhancement in the arterial phase with the variable presentation, and 74.3% had different enhancement patterns between margins and intratumoral parenchyma. Liver lesions in GIST displaying significant, slight hypermetabolism on 18F-FDG PET/CT, marked or heterogeneous gradual enhancement within the intratumoral parenchyma with ring-like enhancement on MRI may denote the diagnosis of liver metastasis. However, GIST liver metastases may also display equal or lower metabolism than liver parenchyma on PET, making small lesions more difficult to diagnose.

Liver magnetic resonance imaging for evaluation of response to treatment after stereotactic body radiation therapy of hepatocellular carcinoma.

BACKGROUNDAlthough stereotactic body radiation therapy (SBRT) is increasingly used, its application has not yet been regulated by the main international guidelines, leaving the decision to multidisciplinary teams.AIMTo assess magnetic resonance imaging (MRI) features of hepatocellular carcinoma (HCC) treated with SBRT, highlighting the efficacy of the treatment and the main aspects of the lesion before and after the procedure.METHODSAs part of a retrospective study, 49 patients who underwent SBRT for HCC between January 2013 and November 2019 were recruited. Each patient underwent a pre-treatment MRI examination with a hepatospecific contrast agent and a similar follow-up examination within 6 mo of therapy. In addition, 22 patients underwent a second follow-up examination after the first 6 mo. The following characteristics were analysed: Features analysed compared to pre-treatment MRI examination, presence or absence of infield and outfield progression, ring-like enhancement, signal hyperintensity in T2-weighted sequences in the perilesional parenchyma, capsular retraction, and "band" signal hypointensity in T1-weighted gradient echo fat saturated sequences obtained during hepatobiliary excretion.RESULTSSignal hyperintensity in the T2-weighted sequences showed a statistically significant reduction in the number of lesions at the post-SBRT first control (P = 0.0006). Signal hyperintensity in diffusion-weighted imaging-weighted sequences was decreased at MRI first control (P < 0.0001). A statistically significant increase of apparent diffusion coefficient values from a median of 1.01 to 1.38 at the first post-control was found (P < 0.0001). Capsular retraction was increased at the late evaluation (P = 0.006). Band-like signal hypointensity in the hepatobiliary phase was present in 94% at the late control (P = 0.006). The study of the risk of outfield progression vs infield progression revealed a hazard ratio of 9.CONCLUSIONThe efficacy of SBRT should be evaluated not in the first 6 mo, but at least 9 mo post-SBRT, when infield progression persists at very low rates while the risk of outfield progression increases significantly.