Japanese clinical practice guidelines for vascular anomalies 2017.

The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety, and systematizing treatment, employing evidence-based medicine (EBM) techniques and aimed at improvement of the outcomes. Clinical questions (CQs) were decided based on the important clinical issues. For document retrieval, key words for literature searches were set for each CQ and literature published from 1980 to the end of September 2014 was searched in Pubmed, Cochrane Library, and Japana Centra Revuo Medicina (JCRM). The strengths of evidence and recommendations acquired by systematic reviews were determined following the Medical Information Network Distribution System (MINDS) technique. A total of 33 CQs were used to compile recommendations and the subjects included efficacy of resection, sclerotherapy/embolization, drug therapy, laser therapy, radiotherapy, and other conservative treatment, differences in appropriate treatment due to the location of lesions and among symptoms, appropriate timing of treatment and tests, and pathological diagnosis deciding the diagnosis. Thus, the Japanese Clinical Practice Guidelines for Vascular Anomalies 2017 have been prepared as the evidence-based guidelines for the management of vascular anomalies.

The Value of MRI and Clinical Features in Differentiating Between Cellular and Fibrous Solitary Fibrous Tumors.

OBJECTIVE: The purpose of this study is to evaluate the usefulness of MRI in differentiating between fibrous and cellular solitary fibrous tumors (SFTs). MATERIALS AND METHODS: This retrospective study included 17 patients with histopathologically confirmed SFTs, including 10 patients with fibrous SFTs and seven patients with cellular SFTs. We evaluated the differences between fibrous and cellular SFTs with regard to clinical data and MRI findings, such as tumor margin definition, signal intensity, heterogeneity on T1- and T2-weighted images, presence of capsules, intratumoral cystic changes, flow signal void, perilesional edema, enhancement pattern on dynamic contrast-enhanced MRI (DCE-MRI), and mean apparent diffusion coefficient (ADC) values. RESULTS: Statistically significant differences in fibrous and cellular SFTs were noted with respect to signal intensity on T2-weighted images (p = 0.044, by Fisher exact test) and enhancement patterns on DCE-MRI (p = 0.005, by Fisher exact test). Specifically, on T2-weighted images, five of the fibrous SFTs had high signal intensity, and the other five had signal isointensity, whereas all seven cellular SFTs had high signal intensity. On DCE-MRI, fibrous SFTs tended to show a gradual increase in enhancement, whereas cellular SFTs showed a rapid initial enhancement pattern. The mean (± SD) ADC value for cellular SFTs was 1.39 ± 0.35 × 10-3 mm2/s, whereas that for fibrous SFTs was 1.37 ± 0.48 × 10-3 mm2/s, with no statistically significant difference noted between the two (p = 0.755, by Fisher exact test). CONCLUSION: Fibrous SFTs have nonspecific findings with regard to signal intensity on T2-weighted MR images and enhancement patterns on DCE-MRI, whereas cellular SFTs show high signal intensity on T2-weighted images and rapid initial enhancement on DCE-MRI.

Ultraselective embolization using a 1.7-Fr catheter and soft bare coil for small intestinal bleeding.

OBJECTIVE: We aimed to evaluate the safety and efficacy of embolization using a 1.7-Fr catheter and soft bare coil to treat acute small intestinal bleeding. MATERIAL AND METHODS: Subjects were five consecutive patients who experienced onset of melena with small intestinal bleeding and underwent transcatheter arterial embolization with 1.7-Fr catheters and 0.010-inch detachable bare coils (five procedures in total). Technical success, clinical success, relative post-procedural complications, arterial bleeding source and cause, and relationship between coagulopathy and embolization efficacy were examined by capsule endoscopy. RESULTS: We achieved 100% technical and clinical success for the five transcatheter arterial embolizations. All catheterizations of the vasa recta of the bleeding artery (jejunal artery, n = 2; ileal artery, n = 3) were possible with a 1.7-Fr catheter. We achieved high embolization efficacy in two patients with coagulopathy. No rebleeding, intestinal ischemia, or necrosis was observed on follow-up capsule endoscopy. We confirmed that peptic ulcers/ulcer scars were the cause of bleeding for all patients. CONCLUSION: Embolization with 0.010-inch coils using a 1.7-Fr catheter and catheterization of the vasa recta of bleeding vessels was effective and safe for treating small intestinal bleeding.

Comparison of ADC values in different malignancies of the skeletal musculature: a multicentric analysis.

OBJECTIVE: Diffusion-weighted imaging (DWI) provides essential information regarding tumor composition, such as cellularity and/or perfusion. DWI is helpful in distinguishing between malignant and benign lesions. Malignant intramuscular/soft tissue lesions consist of a wide spectrum of tumors that have different cell counts and matrix. It is presumed that these different tumors have different DWI findings and have different apparent diffusion coefficient (ADC) values. The aim of this study was to analyze DWI findings of different intramuscular malignancies in a multicentric study by using a standardized DWI protocol, and to compare the ADC values acquired. MATERIALS AND METHODS: The data banks of four radiology departments were screened retrospectively for malignant intramuscular tumors. Only lesions that were investigated by MRI (with a 1.5-T scanner) using DWI (multishot EPI sequence with b values of 0 and 1,000 s/mm(2)) were included in the study. Overall, 51 patients (28 women, 23 men; mean age 58.8 ± 16.1 years) with 57 different malignant intramuscular lesions were collected. In every case apparent diffusion constant (ADC) maps were calculated. In 14 patients muscle lymphoma, 11 patients intramuscular metastases from different primary tumors, and in 26 cases several muscle sarcomas were identified. RESULTS: The mean ADC value of the estimated lesions was 1.24 ± 0.53 × 10(-3) mm(2)s(-1), median value, 1.11 × 10(-3) mm(2)s(-1), range, 0.54-2.9 × 10(-3) mm(2)s(-1). The mean ADC value in muscle metastases was 1.28 ± 0.24 × 10(-3) mm(2)s(-1), in muscle lymphoma 0.76 ± 0.14 × 10(-3) mm(2)s(-1), and in muscle sarcomas 1.82 ± 0.63 × 10(-3) mm(2)s(-1). Muscle lymphoma showed statistically significant lower ADC values in comparison to muscle metastases (p = 0.01) and muscle sarcoma (p = 0.001). There was no significant differences between ADC values in muscle metastases and sarcomas (p = 0.48). ADC values in muscle lymphoma were homogeneous with less inter-patient variability and were within a relatively close range. Muscle sarcomas had a broad range of ADC values. CONCLUSION: Intramuscular malignant lesions had different ADC values on DWI. 22.8% of the tumors analyzed had low ADC values, 26.3% moderate, and 50.9% high ADC values. Muscle lymphoma had statistically significantly lower ADC values in comparison to muscle metastases and sarcomas. Muscle sarcomas presented with a broad range of ADC values.

Analysis of passive motion of para- and retropharyngeal structures during swallowing using dynamic magnetic resonance imaging.

The purpose of this study was to analyze passive motion of the para- and retropharyngeal space (PRS) during swallowing using dynamic magnetic resonance imaging (MRI). We conducted a preliminary study involving 30 healthy volunteers who underwent dynamic MRI. Consecutive MRI axial images were obtained by examining the plane parallel to the hard palate at the level of the anterior inferior corner of C2. Anterior displacement of the posterior pharyngeal wall (PPW) was measured as a motion index of pharyngeal contraction. The displacement and internal angle of the bilateral external and internal carotid arteries (ECA and ICA) and the bilateral centroids of the PRS area, as well as the increase in PRS area, were calculated at rest and at maximum pharyngeal contraction. In most participants, the bilateral ECA, ICA, and centroids were anterointernally displaced by pharyngeal contraction. The normalized ECA displacement (r = 0.64, r (2) = 0.41), normalized ICA displacement (r = 0.60, r (2) = 0.37), and normalized centroid displacement (r = 0.43, r (2) = 0.19) were more than moderately positively correlated with the normalized PPW displacement. The normalized PRS area increase (r = 0.35, r (2) = 0.12) was weakly positively correlated with the normalized PPW displacement. These results revealed that PRS area increased as the ECA and ICA were drawn anterointernally via its passive motion by pharyngeal contraction.

Influence of Gd-EOB-DTPA on proton-density fat fraction in the liver using chemical shift-encoded magnetic resonance imaging at 3-T.

Non-alcoholic fatty liver disease (NAFLD) and its advanced stage, non-alcoholic steatohepatitis (NASH), have become increasingly prevalent owing to the rise in metabolic syndromes. Accurate assessment of hepatic fat deposition and inflammation is crucial for diagnosing and managing NAFLD/NASH. We investigated the influence of Gd-EOB-DTPA, (EOB) on proton-density fat fraction (PDFF) measurements using chemical shift-encoded magnetic resonance imaging (CSE-MRI) at 3-T. In total, 431 patients who underwent EOB contrast-enhanced MRI were included. PDFF measurements were obtained from pre- and post-contrast CSE-MRI. Linear regression and Bland-Altman analyses were performed to assess the correlation and agreement between pre- and post-EOB PDFF measurements. Relative enhancement (RE) of the liver was calculated as an EOB uptake index. There was a significant decrease in PDFF following EOB administration compared with the pre-contrast values (P < 0.0001), which was observed across all PDFF ranges (< 10% and ≥ 10%). Linear regression analysis revealed high correlation between pre- and post-EOB PDFF measurements. Bland-Altman analysis indicated a small bias between pre- and post-EOB PDFF values. Subgroup analysis based on RE showed a significant difference in ΔPDFF between patients with high RE (> 120%) and those with lower RE levels. EOB administration resulted in a slight decrease in PDFF measurements obtained using CSE-MRI at 3-T. We were able to generalize and clarify that the PDFF of the liver on 3D CSE-MRI at 3-T was slightly decreased after EOB administration as we used a larger group of patients compared to previous studies.

The relationship between diffusion tensor imaging and the clinical classification of cubital tunnel syndrome.

The purpose of the study was to investigate the relationship between diffusion tensor imaging (DTI) and the clinical classification of cubital tunnel syndrome (CuTS). Ten patients with CuTS (7 men and 3 women; mean age: 52.7 years) and 5 patients without ulnar neuropathy (2 men and 3 women; mean age: 38.0 years) were enrolled in this retrospective study. Fifteen patients were clinically classified into three groups: "Normal", "1 and 2A", and "2B and 3" by an orthopedic surgeon using the modified McGowan stages. DTI was acquired using a 3.0-T MRI. Fractional anisotropy (FA) of the ulnar nerve was measured in slices covering 20 mm proximal to 20 mm distal to ulnar sulcus. Median FA values in each group were compared by Kruskal-Wallis and Steel-Dwass test (P < 0.05). Five patients with CuTS were classified as "1 and 2A" and five patients as "2B and 3". The FA values, proximal 12 mm to the ulnar sulcus were 0.486 ± 0.117, 0.425 ± 0.166 and 0.298 ± 0.0386 in the "Normal", "1 and 2A" and "2B and 3" groups, respectively. The FA values of patients classified as "Normal" were significantly higher than those classified as "2B and 3" (P = 0.0326 in Steel-Dwass test). FA proximal to the ulnar sulcus might be associated to the modified McGowan stages for the clinical classification of CuTS.

Characteristic findings of primary perirenal angiosarcoma on chemical shift subtraction magnetic resonance imaging (CSS-MRI): A case report.

A 70-year-old man with supraglottic carcinoma underwent computed tomography (CT) for staging purposes. A tumor measuring approximately 7 × 10 cm was found incidentally in the left perirenal space. The tumor showed homogeneous high signal intensity on chemical shift subtraction magnetic resonance imaging (CSS-MRI) suggesting the presence of minimal amounts of fat. Five months later, the tumor had grown to approximately 10 × 12 cm with indistinct margins. CSS-MRI showed high signal intensity in the tumor periphery only. The tumor was resected and the pathological diagnosis was angiosarcoma. Angiosarcomas are malignant endothelial vascular neoplasms that are highly invasive to their surroundings. Here we report a case of primary perirenal angiosarcoma that was difficult to differentiate from a dedifferentiated liposarcoma. On CSS-MRI, high signal intensity within a tumor may be a characteristic feature of primary perirenal angiosarcoma.

Corrigendum to "Characteristic findings of primary perirenal angiosarcoma on chemical shift subtraction magnetic resonance imaging (CSS-MRI): A case report" [Radiology Case Reports 19 (2023) 285-289].

[This corrects the article DOI: 10.1016/j.radcr.2023.10.037.].

Effectiveness of 18F-FDG PET/CT in finding lung metastasis from a retroperitoneal paraganglioma.

A 50-year-old woman was diagnosed with iron deficiency anemia on general medical examination. Further, contrast-enhanced abdominal CT and magnetic resonance imaging revealed a large hypervascular mass with internal degeneration and necrosis in the retroperitoneal space. She was referred to our hospital for further evaluation and treatment. Because the paraganglioma was most likely as the imaging diagnosis, 123I-MIBG scintigraphy was performed. It revealed the marked abnormal accumulation in the retroperitoneal lesion indicating the paraganglioma and no other abnormal accumulation was noted. Several plasma catecholamines and their urinary metabolites were normal. On the subsequent 18F-FDG PET/CT, high FDG uptake was found in the retroperitoneal lesion (SUVmax=38). FDG uptake was also found in a small nodule at the base of the lower lobe of the right lung (SUVmax= 9.8). Contrast-enhanced imaging revealed a hypervascular nodule at the base of the right lung, suggesting pulmonary metastasis of a paraganglioma. The abdominal lesion and right lung nodule were excised, and retroperitoneal paraganglioma and pulmonary metastasis were diagnosed based on the pathology findings. In this case, 18F-FDG PET/CT was useful in the search for paraganglioma metastasis. We report a relationship between 123I-MIBG accumulation and 18F-FDG uptake in paraganglioma and review the relevant literature.

A case of metastasizing pleomorphic adenoma in the maxillary bone appearing twenty years after initial resection of pleomorphic adenoma of the hard palate.

Metastasizing pleomorphic adenoma (MPA) is the inexplicable metastasis of a histologically benign pleomorphic adenoma (PA). Approximately 50 cases have been reported. A 62-year-old woman noticed pain in the upper molar area. Her medical history included an operation for PA in the hard palate that was performed 20 years previously. On imaging, four relatively well-defined lesions were demonstrated in the maxillary bone. She underwent an operation for these lesions. Each lesion revealed the same histological features. Morphological findings displayed typical features of PA. Immunohistochemical staining showed that tumor cells of both primary and metastasizing lesions were positive for pleomorphic adenoma gene (PLAG) 1, which is a sensitive marker for PA. Gene fusions involving PLAG1 were examined by reverse transcription-polymerase chain reaction. However, no gene rearrangements of PLAG1 were found. We report here on a case of MPA in the maxillary bone, which appeared 20 years after resection of the primary tumor and review the relevant literature.

Chondrosarcoma arising within a radiation-induced osteochondroma several years following childhood total body irradiation: case report.

Malignant degeneration arising in radiation-induced osteochondromas is extremely rare. We report a case of a 34-year-old man with a chondrosarcoma arising from an osteochondroma of the left posterior eighth rib that developed following total body irradiation received as part of the conditioning regimen prior to bone marrow transplantation at age 8. To our knowledge, this is only the fourth reported case of a chondrosarcoma arising within a radiation-induced osteochondroma and the first case occurring following childhood total body irradiation.

Value of fat-suppressed T2-weighted imaging for predicting short-term pain relief after sclerotherapy for venous malformations in the extremities.

PURPOSE: This study aimed to evaluate the value of fat-suppressed T2-weighted imaging (FS-T2WI) for predicting short-term pain relief after polidocanol sclerotherapy for painful venous malformations (VMs) in the extremities. MATERIALS AND METHODS: This retrospective study included patients with painful VMs in the extremities between October 2014 and September 2021, had their first sclerotherapy without history of surgical therapy, and underwent magnetic resonance imaging before sclerotherapy. Pain relief was assessed 2 months after 3% polidocanol sclerotherapy and was categorized as follows: progression, no change, partial relief, or free of pain. The associations between pain relief and imaging features on FS-T2WI were analyzed. RESULTS: The study included 51 patients. The no change, partial relief, and free of pain groups included 6 (11.8%), 25 (49.0%), and 20 (39.2%) patients, respectively. No patient experienced progressive pain. The lesion diameter was ≤ 50 mm in 13 (65.0%) patients in the free of pain group, whereas it was > 50 mm in all patients in the no change group (p = 0.019). The lesions showed well-defined margin in 15 (75.0%) patients in the free of pain group, whereas they showed ill-defined margin in 5 (83.3%) patients in the no change group (p = 0.034). The most common morphological type was cavitary in the free of pain group (14 [70.0%] patients), whereas there was no patient with cavitary type lesion in the no change group (p = 0.003). Drainage vein was demonstrated in 6 (100%), 22 (88.0%), and 11 (55.0%) patients in the no change, partial relief, and free of pain group, respectively (p = 0.011). CONCLUSION: A lesion size of 50 mm or less, a well-defined margin, a cavitary type, and no drainage vein on FS-T2WI were significant features for predicting short-term pain relief after polidocanol sclerotherapy for painful VMs in the extremities.

Combination of Prednisolone and Calcineurin Inhibitors Prevents Lung Function Decline in Patients with Anti-aminoacyl-tRNA Synthetase Antibody-Positive Polymyositis/Dermatomyositis.

OBJECTIVE: Anti-aminoacyl-tRNA synthetase antibody-positive polymyositis/dermatomyositis-associ ated interstitial lung disease (ARS-ILD) has a good prognosis, with few cases progressing to respiratory failure. This study aimed to determine factors predictive of lung function changes in patients with ARS-ILD. METHODS: We retrospectively studied 49 patients with ARS-ILD treated at Kurume University Hospital Hospital between 2000 and 2018. We followed 30 patients for more than 2 years after prednisolone (PSL) therapy, with or without calcineurin inhibitors (CNIs), evaluating clinical, physiological, computed tomography, pulmonary func tion, and serological data. RESULTS: After treatment for 24 months, no significant differences were noted between clinical parameters and improvement in forced vital capacity (FVC), %FVC, % carbon monoxide diffusing capacity/alveolar volume (%DLCO), and %DLCO/alveolar volume. Conversely, the annual change of %FVC significantly correlated with the Medical Research Council dyspnea scale grade and %FVC at the first visit and treatment. Furthermore, the annual change of %DLCO/VA significantly correlated with the duration from the first visit to treatment initiation. CONCLUSION: Compared with PSL monotherapy, combining PSL and CNI showed greater mitigation of %FVC decline. The time from onset of ARS-ILD to the first visit is critical for preventing a decline in lung function, and as such, patients should be monitored carefully.

Utility of non-contrast-enhanced magnetic resonance imaging in predicting preoperative clinical stage and prognosis in patients with thymic epithelial tumor.

PURPOSE: The purpose of this study was to find useful imaging features on non-contrast-enhanced magnetic resonance imaging (MRI) that can divide patients with thymic epithelial tumor (TET) into clinical stage I-II and III-IV groups under assumption that contrast media are contraindicated. MATERIALS AND METHODS: This retrospective study included 106 patients (median age, 60 years; range, 27-82 years; 62 women) with surgically resected TET who underwent MRI between August 1986 and July 2015. All cases were classified according to the 2015 WHO classification and staged using the eighth edition of the TNM system. Two radiologists independently evaluated 14 categories of MRI findings; the findings in patients with stage I-II were compared with those of patients with stage III-IV using a logistic regression model. Disease-specific survival associated with significant findings was calculated using the Kaplan-Meier method. RESULTS: Univariate analysis showed that stage III-IV patients were more likely to have tumors with an irregular contour, heterogeneity on T1WI, low-signal intensity on T2WI, irregular border with lung, findings of great vessel invasion (GVI) (hereafter, GVI sign), pericardial thickening/nodule, and lymphadenopathy (all, P < 0.01). On multivariable analysis, only two findings, irregular border between tumor and lung (odds ratio [OR], 272.8; 95% CI 26.6-2794.1; P < 0.001) and positive GVI sign (OR, 49.3; 95% CI 4.5-539.8; P = 0.001) remained statistically significant. Patients with one or both features had significantly worse survival (log-rank test, P < 0.001). CONCLUSION: For patients with TET who are unable to receive contrast for preoperative staging, the two image findings of an irregular border between tumor and lung and the positive GVI sign on non-contrast-enhanced MRI could be helpful in determining stage III-IV disease which is associated with a worse survival.

Infantile juvenile xanthogranuloma of the chest wall mimicking mesenchymal hamartoma: report of a case.

Juvenile xanthogranuloma (JXG) is essentially a benign neoplasm arising from any site on the body; however, there has so far been only one report of JXG located on the chest wall involving a rib. This report presents a rare case finally diagnosed as JXG based on histopathological and immunohistochemical examinations.

Cerebrotendinous xanthomatosis with radiological abnormalities of the chest.

A 55-year-old man with mental retardation and calcaneal tendon thickening was referred for a suspected genetic disease. His serum cholestanol was elevated and genetic analysis of his blood cells for CYP27A1 revealed a homozygous missense mutation. We diagnosed him with cerebrotendinous xanthomatosis (CTX). Chest radiography revealed diffuse micronodular and reticular opacities. Histological findings obtained from the transbronchial lung biopsy revealed foamy macrophages and multinucleate giant cells with marked lipid crystal clefts. Although there are few reports of pulmonary lesions in CTX, we concluded from the radiological and histopathological findings that the pulmonary lesions were indeed caused by the CTX. The patient was treated with chenodeoxycholic acid. His neurological findings and calcaneal tendon thickening were unchanged; however, his serum cholestanol and radiological abnormalities of the chest decreased.

l-carnitine supplementation vs cycle ergometer exercise for physical activity and muscle status in hemodialysis patients: A randomized clinical trial.

Serum carnitine is decreased in hemodialysis patients, which induces muscle atrophy. Thus, we examined the different effects of l-carnitine and exercise on exercise activity and muscle status in hemodialysis patients. Twenty patients were divided into l-carnitine and cycle ergometer groups and were followed for 3 months. Muscle and fat mass, physical activities, and muscle status were evaluated by an impedance, physical function test, and magnetic resonance imaging, respectively. The l-carnitine significantly increased muscle mass (P = .023) and thigh circumference (P = .027), decreased fat mass (P = .007), and shortened chair stand-up time (P = .002) and 10-m walk test (P = .037). The fat fraction was improved by the l-carnitine (P = .047). Compared with the exercise group, l-carnitine improved the changes in 10-m walk test (P = .026), chair stand-up time (P = .014), and thigh circumference (P = .022). Baseline fibroblast growth factor-21 and myostatin levels predicted the l-carnitine-associated changes in exercise activities. l-carnitine, rather than exercise, improved physical activity and muscle status in hemodialysis patients.