A case of a blind-ending right ureter first identified intraoperatively during total laparoscopic hysterectomy for uterine adenomyosis and fibroids in a woman with severe ipsilateral kidney hypoplasia.

Detecting ureteral injury-a serious intraoperative and postoperative complication of hysterectomy-is a high level of difficulty if ureteral malformations are not identified preoperatively. A 39-year-old Japanese woman underwent total laparoscopic hysterectomy for uterine adenomyosis. During surgery, ureteral peristalsis was absent on the right side, whereas the left ureter was visible under the peritoneum. Transabdominal ultrasonography failed to locate the right kidney, with cystoscopy showing no flow from the right ureteral orifice. The ureteral tunnel was identified by tracing the vascular path of the internal iliac and uterine arteries, confirming that the corded tissue was the right ureter. Postoperative contrast-enhanced CT revealed severe right kidney atrophy with no contrast effect or connected ureter. Based on intraoperative laparoscopy and postoperative examination, a diagnosis of a unilateral ureteral scar with a proximal blind end and ipsilateral renal dysgenesis was established, marking the first reported instance of endoscopic visualization of this anomaly. In addition to normal pelvic anatomy, surgeons must consider embryological abnormalities to prevent intraoperative complications.

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.

Precise differentiation between jugular foramen paragangliomas and metastases: utility of diffusion-weighted and dynamic contrast-enhanced magnetic resonance imaging.

PURPOSE: This study tested the utility of diffusion-weighted imaging (DWI) and dynamic contrast-enhanced imaging (DCE-MRI) in differentiating paragangliomas and metastases in the jugular foramen in combination with conventional imaging. METHODS: Forty-nine consecutive patients with paragangliomas or metastases between January 2015 and April 2022 were included in this retrospective study. All patients had pretreatment DWI and DCE-MRI. Between paragangliomas and metastases, normalized apparent diffusion coefficient (nADCmean) and DCE-MRI parameters were compared along with conventional imaging features (enhancement pattern, presence of flow voids, cystic/necrotic change, and bone erosion). The diagnostic performance was tested using receiver operating characteristic (ROC) analysis. RESULTS: Thirty-five paragangliomas (5 male; median 49 years) and 14 metastases (9 male; median 61 years) were analyzed. The most common 3 primary cancers included 4 lung cancers, 3 breast cancers, and 3 melanomas. The presence of flow void was significantly different between paragangliomas and metastases (21/35 vs 2/14; P = 0.0047) in conventional imaging features, while fractional plasma volume (Vp) was significantly different between the two tumor types (median 0.46 vs 0.19; P < 0.001) in DWI and DCE-MRI parameters. The areas under the ROC curves (AUCs) of the presence of flow void and Vp were 0.72 and 0.93, respectively. The AUC of the combination of the presence of flow void and Vp was 0.95 and significantly improved compared to that of the presence of flow void (P < 0.001). CONCLUSION: Adding DCE-MRI to the head and neck protocol can aid in the precise differentiation between jugular foramen paragangliomas and metastases.

The necessity of gait evaluation on the 7th day after tap tests for the idiopathic normal pressure hydrocephalus patients.

BACKGROUND: A tap test is established as an evaluation method to indicate shunt surgery for hydrocephalus, especially idiopathic normal pressure hydrocephalus (iNPH). The timing of gait assessment after the test is still controversial, while some studies reported the effectiveness of the gait evaluation up to 2nd day after tap tests. Our study explored whether the gait evaluation on the 7th day after a tap test is necessary. METHODS: We retrospectively evaluated 129 consecutive cases with possible iNPH who performed gait assessment on all 1st, 3rd, and 7th days after tap tests between May 2020 and February 2022. We reviewed the following items of the patients: age, sex, modified Rankin scale, iNPH grading scale, Mini-Mental State Evaluation (MMSE), and neurological imaging. The number of probable iNPH patients who improved their gait each day after the test was analyzed. We also assessed the number of definite iNPH patients and revealed the background characteristics of the patients who showed gait improvement on the 7th day after the tests. RESULTS: Of the 129 patients who met our inclusion criteria, 57 were judged as probable iNPH on the 1st day, 28 were new on the 3rd, and 23 were new on the 7th. The number of probable iNPH patients up to the 7th day after tests was significantly more extensive than that of those up to the 3rd (108 [83.7%] vs. 85 [65.9%]; 95% confidence interval [CI], p < 0.0001). The number of definite iNPH patients was also significantly more prominent when the evaluation after the tests was performed on all of the 1st, the 3rd, and the 7th days than just on the 1st (72 vs. 42; 95% CI, p = 0.00016) or both of the 1st and the 3rd (72 vs. 61; 95% CI, p = 0.00074). No statistically significant difference existed in the patients' backgrounds except for the pre-tap test MMSE. CONCLUSION: Gait evaluation on the 7th day after tap tests, in addition to the first few days, may reduce the number of iNPH patients who miss the opportunity of getting beneficial treatment.

Combined histological and DNA methylome profiling approaches may provide insights into the pathophysiology of ovarian endometriomas.

Purpose: To test the theory that invaginated ovarian surface epithelium and endometrial implants on the ovary form ovarian endometriomas. Methods: Adhesion sites of ovarian endometrioma on the peritoneum and consecutive ovarian endometrioma cyst wall, called non-adhesion sites, were histologically examined. DNA methylomes of the adhesion sites, non-adhesion sites, and blueberry spots were compared with those of ovary, endometrium, and peritoneum. Results: The non-adhesion sites showed an ovarian surface epithelium-like structure near the adhesion site, which continued to a columnar epithelium-like structure. Calretinin staining was strong in the ovarian surface epithelium-like structure but weak in the columnar epithelium-like structure. Estrogen receptors were absent in the ovarian surface epithelium-like structure, but present in the columnar epithelium-like structure. The adhesion sites had endometrial gland-like structures that expressed estrogen receptors. Analyses of DNA methylomes classified the non-adhesion sites and ovaries into the same group, suggesting that ovarian endometriomas originate from the ovarian surface epithelium. The adhesion sites, blueberry spots and peritoneum were classified in the same group, suggesting that the adhesion sites and blueberry spots originate from the peritoneum. Conclusions: The present results support the invagination theory. Ovarian endometriomas consist of invaginated ovarian surface epithelium with celomic metaplasia and endometrium implants on the peritoneum.

Imaging of sleep disorders in pre-Parkinsonian syndromes.

PURPOSE OF REVIEW: Neuroimaging has been advanced in the last years and enabled clinicians to evaluate sleep disorders, especially isolated rapid eye movement sleep disorder (iRBD), which can be seen in alpha-synucleinopathies. iRBD is the best prodromal clinical marker for phenoconversion to these neurodegenerative diseases. This review aims to provide an update on advanced neuroimaging biomarkers in iRBD. RECENT FINDINGS: Advanced structural MRI techniques, such as diffusion tensor imaging and functional MRI, neuromelanin-sensitive MRI, and scintigraphic neuroimaging such as cholinergic PET, dopamine transporter imaging - single-photon emission computerized tomography, perfusional single-photon emission computerized tomography, and cardiac metaiodobenzylguanidine can provide diagnostic and prognostic imaging biomarkers for iRBD, in isolation and more robustly when combined. SUMMARY: New advanced neuroimaging can provide imaging biomarkers and aid in the appropriate clinical assessment and future therapeutic trials.

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.

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.

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.

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.

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.

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.

Imatinib-induced pancreatic hypertrophy in patients with gastrointestinal stromal tumor: Association with overall survival.

OBJECTIVES: To investigate the frequency of imatinib-induced pancreatic complications and determine whether these are survival prognostic factors in patients with gastrointestinal stromal tumor (GIST). METHODS: This retrospective multicenter study included patients with histopathologically diagnosed GIST treated with imatinib who underwent computed tomography (CT) within 100 days before (pretreatment CT) and 500 days after (post-treatment CT) imatinib initiation (January 2004-December 2019). Forty-eight patients (63.0 ± 12.1 years, 30 men) were included. Two blinded radiologists independently measured pancreatic volumes. Pancreatic volume on pretreatment CT was compared with that of the control (within 1 year prior to pretreatment CT) and the first two post-treatment CTs using paired t-tests. Thresholds for pancreatic hypertrophy and atrophy were defined using a log-rank test. The prognostic importance of pancreatic hypertrophy was further analyzed using multivariate Cox proportional hazard regression models. RESULTS: Pancreatic volume was significantly higher for the first post-treatment CT than pretreatment CT (71.5 cm3 vs. 67.4 cm3, P = .027), whereas no significant difference was observed between the pretreatment and control CTs. Optimal thresholds for pancreatic hypertrophy and atrophy were defined as an 22% increase and 30% decrease and found in 20 and three patients, respectively. Pancreatic hypertrophy was significantly associated with reduced survival [hazard ratio = 2.9 (95% confidence interval, 1.3-6.5), P = .0088]. No patients showed serum lipase elevation, nor were they suspected of having acute pancreatitis. CONCLUSION: There was frequent asymptomatic pancreatic swelling in patients with GIST after imatinib treatment, and a ≥22% increase in pancreatic volume was a predictor of reduced survival.

Radiological features of head and neck mammary analogue secretory carcinoma: 11 new cases with a systematic review of 29 cases reported in 28 publications.

PURPOSE: This retrospective study aimed to investigate the radiological features of head and neck mammary analogue secretory carcinoma (MASC) and systematically review previous publications and 11 new cases. METHODS: We included patients with pathologically proven MASCs with preoperative CT or MRI images, including 11 patients from our hospital and 29 patients from 28 publications extracted after screening 645 abstracts. Two board-certified radiologists reviewed and evaluated all radiological images. The frequency of metastasis during the follow-up period in tumors with well- and ill-defined margins was compared using a Fisher's exact test. RESULTS: The median age at diagnosis was 52.5 years (range, 7-78 years; 20 males). Of the 40 patients, those in their 50 s were the most common (10/40, 25.0%), and the main tumor site was the parotid gland (27/40, 42.5%). Characteristic radiological features included high intensity on T1WI in the cystic components and tumors frequently showed "papillary and cystic," which showed a projection into the cystic components, or "non-papillary and cystic" morphology, which did not show projection of the solid components. Tumor metastasis was found in 10/35 patients (28.6%) during the follow-up period, with a significant difference in frequency between the tumors with well- and ill-defined margin (well-defined (4/26) vs. ill-defined (6/9); P = 0.0074). CONCLUSION: MASCs are newly recognized malignant tumors. Characteristic T1WI high intensity on MRI and predominant cystic morphology may reflect its unique histological profile. Ill-defined tumor margin status was related to frequent metastasis. Awareness of these characteristic radiological features can assist radiologists in better detection of this novel entity.

Atypical imaging findings of presumed stroke-like migraine attacks after radiation therapy syndrome in the brainstem.

The authors present an atypical case of presumed stroke-like migraine attacks after radiation therapy (SMART) syndrome in the brainstem. A 29-year-old male, who had been treated with resection and subsequent craniospinal radiation for posterior fossa medulloblastoma 21 years before, presented with subacute progressive left hemiparesis evolving over 4 days. Hematological findings, cerebrospinal fluid (CSF), and electroencephalogram (EEG) were unremarkable. Magnetic resonance imaging (MRI) showed a round area of hyperintense FLAIR signal centered within the pons associated with central restricted diffusion, peripheral enhancement, and small paramagnetic low susceptibility signal foci consistent with petechial hemorrhage. Positron emission tomography (PET), perfusion MRI, and MR spectroscopy revealed no evidence of tumor recurrence. The diagnosis of SMART syndrome is presumed from the conventional and advanced imaging findings, clinical history, and clinical course.

Texture analysis of T2-weighted MRI predicts SDH mutation in paraganglioma.

PURPOSE: Texture analysis can quantify sophisticated imaging characteristics. We hypothesized that 2D textures computed with T2-weighted and post-contrast T1-weighted MRI can predict succinate dehydrogenase (SDH) mutation status in head and neck paragangliomas. METHODS: Our retrospective study included 21 patients (1 to 4 tumors/patient) with 24 pathologically proven paragangliomas in the head and neck. Fourteen lesions (58%) were SDH mutation-positive. All patients underwent T2-weighted and post-contrast T1-weighted MRI sequences. Three 2D texture features of dependence non-uniformity normalized (DNN), small dependence high gray level emphasis (SDHGLE), and small dependence low gray level emphasis (SDLGLE) were calculated. Computed textures between SDH mutants and non-mutants were compared using Mann-Whitney U test. Area under the receiver operating characteristic (AUROC) curve was used to quantify the predictive power of each texture. RESULTS: Only T2-based SDLGLE was statistically significant (p = 0.048), and AUROC was 0.71. Diagnostic accuracy was 70.8%. CONCLUSION: 2D texture parameter of T2-based SDLGLE predicts SDH mutation in head and neck paragangliomas. This noninvasive technique can potentially facilitate further genetic workup.

Forward-projected Model-based Iterative Reconstruction SoluTion in Temporal Bone Computed Tomography: A Comparison Study of All Reconstruction Modes.

OBJECTIVE: Identify appropriate reconstruction modes of Forward-projected model-based Iterative Reconstruction SoluTion (FIRST) in temporal bone computed tomography (CT) and investigate the contribution of spatial resolution and noise to the visual assessment. METHODS: Sixteen temporal bone CT images were reconstructed. Two blinded radiologists assessed 8 anatomical structures and classified the visual assessment. These visual scores were compared across reconstruction modes among each anatomical structure. Visual scores and contrast-to-noise ratio, noise power spectrum (NPS) at low (NPSLow) and high (NPSHigh) spatial frequencies, and 50% modulation transfer function relationships were evaluated. RESULTS: Visual scores differed significantly for the stapedius muscle and osseous spiral lamina, with CARDIAC SHARP, BONE, and LUNG modes for the osseous spiral lamina scoring highest. Visual scores significantly positively correlated with NPSLow, NPSHigh, and 50% modulation transfer function but negatively with contrast-to-noise ratio. CONCLUSIONS: Modes providing higher spatial resolution and lower noise reduction showed an improved visual assessment of CT images reconstructed with FIRST.

A controlled clinical trial comparing potent progestins, LNG-IUS and dienogest, for the treatment of women with adenomyosis.

PURPOSE: To evaluate the efficacy of two progestins, levonorgestrel intrauterine system (LNG-IUS) and dienogest (DNG), for adenomyosis. METHODS: This study enrolled 157 women with adenomyosis, randomized to either LNG-IUS (n = 76) or DNG (n = 81) groups as a controlled clinical trial for 72 months. Participants were classified by three different localizations of adenomyosis: diffuse, focal, and extrinsic. VAS (Visual analog scale) score, days, and amount of uterine bleeding were assessed. Uterine volume or bone mineral density (BMD) were measured by three-dimensional ultrasonography or dual-energy X-ray absorptiometry. RESULTS: LNG-IUS and DNG comparably reduced pain scores in patients with adenomyosis. With regard to pain control, DNG offered greater efficacy than LNG-IUS in 3 months of treatment. In all types of adenomyosis, the days of bleeding after 12 months with DNG were significantly decreased compared to those with LNG-IUS. The decrease of whole uterine body was transient in any subtypes. A comparable decrease in BMD due to age-related changes in both groups was observed. CONCLUSIONS: LNG-IUS and DNG could be useful for long-term management of adenomyosis. In terms of durations of uterine bleeding, DNG was superior to LNG-IUS for 6 years.

Computed tomography findings of early-stage TAFRO syndrome and associated adrenal abnormalities.

OBJECTIVES: To compare CT findings of early (within 3 weeks post-onset)- and later (within 1 month before or after diagnostic criteria were satisfied, and later than 3 weeks post-onset) stage thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome. METHODS: Between 2014 and 2019, 13 patients with TAFRO syndrome (8 men and 5 women; mean age, 54.9 years) from nine hospitals were enrolled. The number of the following CT findings (CT factors) was recorded: the presence of anasarca, organomegaly, adrenal ischaemia, anterior mediastinal lesion, bony lesion, and lymphadenopathy. Records of adrenal disorders (adrenomegaly, ischaemia, and haemorrhage) throughout the disease course were also collected. Differences in CT factors at each stage were statistically compared between remission and deceased groups. RESULTS: Para-aortic oedema and mild lymphadenopathy were observed in all patients, whereas pleural effusion, ascites, and subcutaneous oedema were found in 5/13, 7/13, and 7/13 cases, respectively, at the early stage. CT factors at the early stage were significantly higher in the deceased than in the remission group (mean, 11 vs 6.5; p = 0.04), while they were nonsignificant at the later stage. Adrenal disorders were present in 7/13 cases throughout the course including 6 of adrenomegaly and 4 of ischaemia at the early stage. CONCLUSIONS: Para-aortic oedema and mild lymphadenopathy are most common at the early stage. Anasarca, organomegaly, lymphadenopathy, and adrenal disorders on early-stage CT are useful for unfavourable prognosis prediction. Moreover, adrenal disorders are frequent even at the early stage and are useful for early diagnosis of TAFRO syndrome. KEY POINTS: • CT findings facilitate early diagnosis and prognosis prediction in TAFRO syndrome. • Adrenal disorders are frequently observed in TAFRO syndrome. • Adrenal disorders are useful for differential diagnosis of TAFRO syndrome.