Case report: Characteristics and nature of primary cardiac synovial sarcoma.

Primary malignant cardiac tumors rarely occur, and cardiac synovial sarcoma (SS) is especially rare among such tumors. Herein, we present the case of a 35-year-old female with primary cardiac SS treated with surgery, chemotherapy, and radiotherapy. She presented with chest symptoms and underwent imaging examinations. A cardiac tumor was suspected, and an open biopsy was performed. The pathological findings suggested cardiac SS. Next, we performed a resection, and the tumors persisted at a macroscopic level. Immunohistochemistry was negative for SS18-SSX and positive for the SSX C-terminus and cytokeratin CAM5.2, a reduction of SMARCB1/INI1 was observed, and fluorescence in situ hybridization showed positive SS18 split staining. Owing to the FNCLCC grade 3 tumor and R2 margins, adjuvant chemotherapy with ifosfamide, doxorubicin, and radiotherapy was initiated, and the patient was diagnosed with cardiac SS. The differences in patients with cardiac SS compared with general SS include male predominance, larger tumor size, and poorer prognosis. Pathological findings of immunohistochemistry and fluorescence in situ hybridization were found to be more reliable than imaging findings for a correct diagnosis. Additionally, because incomplete resection is frequently performed, adjuvant therapy, including chemotherapy and radiation therapy, may be performed. The findings indicate that multiple therapies, including surgery, chemotherapy, and radiotherapy, are essential treatment strategies for improving the prognosis of patients with cardiac SS.

A Rare Clinical Presentation With a Difficult Imaging Diagnosis of an Intra-articular Clear Cell Sarcoma of the Knee.

ABSTRACT: Clear cell sarcoma, a highly aggressive cell sarcoma with melanotic differentiation, typically occurs in the tendon and aponeuroses of the lower extremities and rarely develops in the intra-articular region. Herein, we present an extremely rare case of clear cell sarcoma originating from the intra-articular knee and suspected as benign tumors due to the benign-looking findings on MRI and PET/CT. The image results in our case were completely consistent with a tenosynovial giant cell tumor, resulting in the misdiagnosis. For differential diagnosis, especially malignant melanoma, histopathology, including IHC and FISH, was indispensable.

Synovial chondromatosis: Rare involvement of metacarpophalangeal joint and diagnostic challenges in MR imaging.

Synovial chondromatosis (SC) is a rare benign tumor of the cartilaginous tissue that frequently affects large joints intra-articularly. Characteristic imaging findings were reported for the diagnosis of SC. Herein, we report a case of SC of the metacarpophalangeal joint with atypical MRI findings. Considering the clinical presentation, erosion, absence of calcification on X-ray and CT, and low intensity on short tau inversion recovery (STIR), tenosynovial giant cell tumor was the initially diagnosis. However, histopathological findings revealed SC with fibrosis, which was confirmed using Elastica Van Gieson (EVG) staining. In general, low intensity on STIR in SC indicate calcification; however, it can also represent fibrosis in SC. In this case, the diagnosis of SC using MRI was limited highlighting the importance of histopathological findings for an accurate diagnosis of SC.

Indolent Multinodular Synovial Sarcoma of Peripheral Nerves Mimicking Schwannoma: A Case Report and Literature Review.

BACKGROUND/AIM: Most cases of synovial sarcoma (SS) are aggressive and large-sized; only few show indolent behavior, having a small size. Nerves are rare sites of SS occurrence. An atypical case of SS can lead to its misdiagnosis as a benign tumor and delay its treatment. CASE REPORT: Here, we report a case of primary SS of indolent multinodular synovial sarcoma of peripheral nerves. Considering the clinical and imaging findings at the first visit, we suspected a benign tumor and continued careful follow-up. Three years later, marginal resection was performed and SS was suspected. We then performed an additional wide resection using a free flap. Histopathologically, the proximal tumor showed a diffuse proliferation of spindle cells without pleomorphism, whereas the distal tumor showed a similar histology with more hypercellularity. Additional wide-resection specimens showed remnant tumors derived from the peripheral nerve. Immunohistochemistry (IHC) showed positive staining for SS18:SSX and SSX in both tumors and fluorescence in situ hybridization showed positive staining for the SS18 split in both tumors. Finally, SS of the peripheral nerve was diagnosed. Owing to FNCLCC grade 2 tumor and tumor size, adjuvant chemotherapy was not performed. CONCLUSION: In cases of SS or other sarcomas with atypical clinical courses, with imaging findings mimicking benign tumors, we recommend marginal resection along with pathological examination for correct diagnosis.

Inter-observer agreement and accuracy of LI-RADS v2018 for differentiating tumor in vein from bland thrombus using gadoxetic acid-enhanced magnetic resonance imaging.

PURPOSE: To assess inter-observer agreement and accuracy of LI-RADS v2018 for differentiating tumor in vein (TIV) from bland thrombus on gadoxetic acid-enhanced magnetic resonance imaging (Gx-MRI). Secondarily, to determine whether a multi-feature model improves accuracy compared to LI-RADS. METHODS: We retrospectively identified consecutive patients at risk for hepatocellular carcinoma with venous occlusion(s) reported on Gx-MRI. Five radiologists independently classified each occlusion as TIV or bland thrombus using the LI-RADS TIV criterion (enhancing soft tissue in vein). They also evaluated imaging features suggestive of TIV or bland thrombus. Intra-class correlation coefficient (ICC) was calculated for individual features. A multi-feature model was developed based on consensus scores of features with > 5% consensus prevalence and > 0.40 ICC. Sensitivity and specificity of the LI-RADS criterion and of the cross-validated multi-feature model were compared. RESULTS: Ninety-eight patients with 103 venous occlusions (58 TIV, 45 bland thrombus) were included. The LI-RADS criterion provided 0.63 ICC and, depending on the reader, 0.62-0.93 sensitivity and 0.87-1.00 specificity. Five other features had > 5% consensus prevalence and > 0.40 ICC, including three LI-RADS suggestive features and two non-LI-RADS features. The optimal multi-feature model incorporated the LI-RADS criterion and one LI-RADS suggestive feature (occluded or obscured vein contiguous with malignant parenchymal mass). After cross-validation, the multi-feature model did not improve sensitivity or specificity compared to the LI-RADS criterion (P = 0.23 and 0.25, respectively). CONCLUSION: Using Gx-MRI, the LI-RADS criterion for TIV provides substantial inter-observer agreement, variable sensitivity, and high specificity for differentiating TIV from bland thrombus. A cross-validated multi-feature model did not improve diagnostic performance.

Stereotactic body radiotherapy for metachronous multisite oligo-recurrence: a long-surviving case with sequential oligo-recurrence in four different organs treated using locally radical radiotherapy and a review of the literature.

Stereotactic body radiotherapy (SBRT) for oligometastases represents a recent trend in radiation oncology. While abundant data are available regarding the use of SBRT for the treatment of lung or liver oligometastases from various retrospective series and prospective trials, relatively little information has been accumulated for the treatment of oligometastases at sites other than the lungs and liver, particularly for sequential oligometastases in multiple organs. Oligometastases with primary lesions controlled is called "oligo-recurrence." We describe herein the case of a lung cancer patient who developed repeated oligo-recurrence at multiple sites that were each controlled by radical radiotherapy and achieved long-term survival and discuss the merits of locally aggressive radiotherapy for this type of disease condition with reviewing the literature. Although further investigation should be undertaken to clarify the benefits, objectives, and methods of SBRT for the treatment of oligometastases, we believe utilization of SBRT may be worthwhile for patients with remote metastases who hope for treatment to acquire better local control and possible longer survival.

Large prostate motion produced by anal contraction.

BACKGROUND AND PURPOSE: The aim of this study was to define the effects of voluntary anal contraction on prostate motion in an experimental setting. MATERIALS AND METHODS: Thirty-eight patients (median age, 76 years) with prostate cancer underwent thin-slice computed tomography (CT) in the vicinity of the prostate before and after active anal contraction. Three-dimensional displacement of the pelvis and prostate was measured. RESULTS: Mean (±standard deviation, SD) overall displacement of the prostate due to anal contraction was 0.3±1.4 mm to the right, 9.3±7.8 mm to the anterior, and 5±4 mm to the cranial direction. Mean displacement of the pelvis was 0.5±1.8 mm to the right, 4.1±7.1 mm to the anterior, and 1±3 mm to the cranial direction. Mean displacement of the prostate relative to the pelvis was 0.1±1.1 mm to the left, 5.2±3.3 mm to the anterior, and 4±4 mm to the cranial direction. CONCLUSIONS: Voluntary anal contraction within an experimental setting induces large prostate and bone motion, mainly in the anterior and cranial directions. The frequency and magnitude of actual anal contractions during radiotherapy for prostate cancer need to be determined.

Optimal iodine dose for 3-dimensional multidetector-row CT angiography of the liver.

PURPOSE: To clarify the optimal iodine dose of contrast material for 3-dimensional multidetector-row CT angiography (3D-MDCTA) of the venous vasculature of the liver using volume rendering technique. MATERIALS AND METHODS: This study included 103 patients who were randomly assigned to 5 contrast-enhanced MDCT protocol groups with different body-weight-tailored doses of contrast material: 500, 600, 630, 650, and 700 mgI/kg body weight. The arterial, portal, and hepatic parenchymal phases were obtained to evaluate enhancement values of the aorta, portal vein, and hepatic vein. Visualization of the portal and hepatic veins on the volume-rendering images of 3D-MDCTA was evaluated using a 5-point grade. Dunnett's test was used to compare the mean enhancement value and mean grades of image quality (700 mgI/kg dose group was control). RESULTS: The mean enhancement values of portal and hepatic vein in the group with 500 and 600 mgI/kg were significantly lower than those of the control group. During visual assessment, a significantly lower mean grades were observed in 500 mgI/kg groups for the portal vein, and 500 and 600 mgI/kg groups for hepatic vein. There were no significant intergroup differences in mean enhancement values and visual assessment among the groups using 630 mgI/kg or more. CONCLUSION: Iodine doses of 630 mgI/kg was recommended for 3D-MDCTA.

Outcome of hypovascular hepatic nodules revealing no gadoxetic acid uptake in patients with chronic liver disease.

PURPOSE: To elucidate the natural history of hypovascular nodules that appear hypointense on hepatocyte-phase gadoxetic acid-enhanced MR images by focusing on hypervascularization over time. MATERIALS AND METHODS: In this study, 135 hypovascular nodules revealing no gadoxetic acid uptake in 53 patients were examined. All nodules were retrospectively examined using serial follow-up computed tomography (CT) and MRI examinations until hypervascularity was observed on arterial-phase dynamic CT or gadoxetic acid-enhanced MR images, or on CT during hepatic arteriography. Logistic regression analysis was used to investigate the association between hypervascularization and MR findings including a presence of fat assessed by a signal drop on opposed-phase T1-weighted images. RESULTS: Of the 135 nodules, 16 underwent hypervascularization. The size of the nodules and the presence of fat in the nodules were independent indicators of hypervascularization. The 1-year cumulative risk of hypervascularization was 15.6%. This risk was significantly increased in the case of nodules >10 mm (37.6%, P < 0.01) and fat-containing nodules (26.5%, P < 0.01). CONCLUSION: Hypovascular nodules that appear hypointense on hepatocyte-phase gadoxetic acid-enhanced MR images may progress to conventional hypervascular hepatocellular carcinoma. Nodules more than 10 mm in diameter and containing fat are at high risk for developing hypervascularization.

Distinguishing hepatic metastasis from hemangioma using gadoxetic acid-enhanced magnetic resonance imaging.

PURPOSE: To retrospectively determine the findings of gadoxetic acid-enhanced magnetic resonance imaging (MRI) to distinguish hemangioma and metastasis of the liver. MATERIALS AND METHODS: The University's ethics committee approved this retrospective study. We assessed 45 patients without chronic liver disease who underwent gadoxetic acid-enhanced MRI. Diagnosis of 58 metastases and 47 hemangiomas was confirmed using histopathology or multimodality evaluation. Two radiologists independently assessed the following MRI findings of metastasis and hemangioma: their appearance on T2-weighted images (T2WI) and dynamic contrast enhancement patterns after gadoxetic acid administration. The metastasis and hemangioma findings were compared using Fisher exact test. The lesion-to-liver signal intensity ratio on hepatocyte phase was compared using the Mann-Whitney U test. Multivariate analysis was performed to identify independent imaging findings distinguishing the 2 diseases. Receiver operating characteristic analysis was used to estimate the diagnostic ability of gadoxetic acid-enhanced MRI to distinguish metastasis from hemangioma. RESULTS: The lesion-to-liver signal intensity ratio was comparable in both diseases. Peripheral-dot enhancement, ring-like, geographic, and moderate late-phase enhancement, rapid contrast filling, and bright signal on T2WI could differentiate between the 2 diseases. In multivariate analysis, bright signal on T2WI (94%-98% in hemangioma and 13%-25% in metastasis) and ring-like enhancement (4% in hemangioma and 58%-60% in metastasis) were the independent findings suggesting hemangioma and metastasis, respectively. The areas under the receiver operating characteristic curves to distinguish metastasis from hemangioma were 0.95 and 0.98 for Reader 1 and 2, respectively. CONCLUSION: Reliable findings to distinguish hepatic metastasis from hemangioma on gadoxetic acid-enhanced MRI were ring-like enhancement on arterial-phase images and bright signal on T2WI.

Double-dose gadoxetic Acid-enhanced magnetic resonance imaging in patients with chronic liver disease.

OBJECTIVES: determine the effect of double-dose gadoxetic-acid (Gd-EOB-DTPA) on lesion-liver contrast ratio in arterial- and hepatocyte-phase images and arterial-phase image quality in patients with chronic liver disease. MATERIALS AND METHODS: the ethics committee at our institute approved this study. This study included 28 patients (13 with Child-Pugh class A and 15 with class B) with 54 hepatocellular carcinomas. All patients received the standard Gd-EOB-DTPA dose (0.025 mmol/kg bodyweight) and double dose (0.050 mmol/kg bodyweight). The lesion-liver contrast ratio was evaluated in arterial- and hepatocyte-phase images. The artifacts in arterial-phase images were evaluated with a 4-point scale. Wilcoxon signed-rank test were used for comparisons. RESULTS: the hepatocyte-phase lesion-liver contrast ratio after the double dose was significantly higher than that after the standard dose in patients with Child-Pugh class B disease(standard dose vs. double dose; 0.20 ± 0.16 vs. 0.25 ± 0.17; P < 0.0001); however, the ratio after both the standard and double doses was equivalent in patients with Child-Pugh class A disease (0.35 ± 0.18 vs. 0.35 ± 0.14; P = 0.3038). The double dose significantly increased the arterial-phase lesion-liver contrast ratio (0.34 ± 0.19 vs. 0.58 ± 0.33; P < 0.0001). The artifacts in the arterial-phase images were more prominent after the standard dose (2.7 vs. 2.4 for reader 1, 2.8 vs. 2.4 for reader 2; P = 0.0195 and 0.0010). CONCLUSIONS: administration of double dose of Gd-EOB-DTPA provided better arterial enhancement of hepatocellular carcinomas in patients with chronic liver disease, and also improved the lesion-liver contrast in hepatocyte-phase images in patients with Child-Pugh class B disease.