Rock on the Heart: A Case of Calcifying Fibrous Tumor Arising From the Pericardium and Literature Review.

Calcifying fibrous tumors are rare benign fibrous tumors that rarely occur in the heart. We report a 33-year-old woman who was found to have a benign pericardial tumor on health checkup, which was highly suspected to be a teratoma in clinical and imaging examination. After cardiac tumor resection, histopathological features showed scattered foci of psammoma bodies or calcification among collagenized fibrous connective tissues and foci of inflammatory cell infiltration in the interstitium. The pathological diagnosis was a calcifying fibrous tumor.

Primary cardiac undifferentiated pleomorphic sarcoma is associated with TP53 mutation during lack of MDM2 amplification, and targeted sequencing analysis reveals potentially actionable targets.

Cardiac undifferentiated pleomorphic sarcoma (UPS) is a rare malignancy. Several studies have revealed frequent MDM2, CDK4, PDFGRA, and KIT amplifications and CDKN2A and CDKN2B deletions. Cases lacking the above copy number alterations may harbor alternative driver mutations; however, little is known about such occurrences. This study was conducted to gain further insights into the molecular features of cardiac UPS using targeted sequencing of 560 cancer-related genes, and fluorescence in situ hybridization and immunohistochemistry of MDM2, CDK4, CDKN2A, TP53, and RB1 in 9 cardiac UPS cases. TP53 mutation or CDKN2A deletion was found in cases lacking MDM2 amplification. Further, p53 overexpression was detected in the case with TP53 mutation, while p16 expression was completely lost in the case with CDKN2A homozygous deletion. p16 overexpression was found in cases with MDM2 and CDK4 amplification but without CDKN2A deletion. Immunohistochemistry of MDM2, CDK4, p53, and p16 is expected to be preliminarily used for gene status analysis. As cardiac UPS and intimal sarcomas are merging into a single spectrum, mutation data for 3 cardiac UPS and 9 intimal sarcomas from the literature, as well as data for 5 cardiac UPS in our study were evaluated, and known recurrently mutated cancer driver genes, including PDGFRB, TP53, ALK, PTCH1, RET, ERBB4, JAK3, GATA1, PIK3CG, and RARA, were identified. Several new potentially actionable mutations, including those in RARA, ALK, PTCH1, RET, ROS1, ABL1, and MET, were also found. These findings improve the molecular understanding of this rare malignancy and are expected to provide a basis for developing precision therapeutics for cardiac UPS and intimal sarcomas.

Cardiac fibroma: A clinicopathologic study of a series of 12 cases.

BACKGROUND: Cardiac fibroma (CF) is a rare tumor that has not been widely reported. This study investigated the clinical findings, histologic features, and differential diagnosis of CF. METHODS: A total of 12 CF cases were studied and reviewed using hematoxylin and eosin (H&E), special staining and immunohistochemical staining. The ALK gene was tested in 4 cases of cardiac fibroma with significant inflammatory cells. Clinicopathological data were retrospectively analyzed and followed up. RESULTS: The cases occurred in six males and six females ranging in age from 0.5 to 55 years (median, 5 years). The tumors were grossly single and solid (1-17 cm; mean 5.6 cm). The clinical signs and symptoms depended largely on the location of the tumor. Microscopically, the CFs observed were composed of monomorphic spindle cells and abundant collagen. The spindle cells demonstrated little or no atypia. The histology of CFs in infants and young children showed some differences from those in adults. Infants and young children with fibromas exhibited cellular types with more inflammatory infiltration. All tumors expressed vimentin markers. Eleven of 12 cases (91.7%) were positive for SMA by immunohistochemistry. ALK immunostaining and ALK-FISH tests showed negative results. Follow-up information was available for all patients. The mean postoperative follow-up was at 3 years (range 2 months-8.8 years). All patients were alive with no evidence of disease. CONCLUSIONS: Our study shows that CFs exhibit a wide morphological spectrum of soft tissue tumors with fibroblastic or myofibroblastic differentiation and/or components. Infants and younger pediatric patients with fibromas have tumors that are more hypercellular and more likely to be misdiagnosed with aggressive or malignant lesions than adults. Finally, the data indicate that CF exhibits benign behavior and that local resection is safe and effective.

CT Findings of Pulmonary Metastases from Primary Cardiac Angiosarcoma.

BACKGROUND: Primary cardiac angiosarcoma is a rare malignancy with high predilection to involve surrounding structures such as pulmonary metastases. We analysed the chest computed tomography (CT) imaging features of patients diagnosed with primary cardiac angiosarcoma with pulmonary metastases in this study. METHODS: This study retrospectively reviewed 12 patients with confirmed primary cardiac angiosarcoma, out of which eight (all men) with pulmonary metastasis were included in the analysis. The patients' age ranged from 17 to 74 (mean: 48) years. CT was performed in all patients with unenhanced, contrast-enhanced, and both scans were done in 1, 3 and 4 patients, respectively. RESULTS: Nodular lesions were observed in 7 patients with multiple solid nodules observed in 6 out of 7 patients. A solitary solid nodule was found in the remaining patient in the upper lobe and apical segment of the right lung with a diameter of 11.7 mm. All solid nodules were distributed along with bronchovascular bundles in the lungs, and their maximum diameter ranged from 2.3 to 19.9 mm. Nodules larger than 10 mm in diameter were heterogeneously enhanced on contrast-enhanced CT images (5/8 patients), whereas those smaller than 10 mm were homogeneously enhanced (3/8 patients). Other imaging features, namely the tree-in-bud pattern, emphysema, pleural effusion, and mediastinal lymph node enlargement, were observed in 4, 3, 3, and 2 patients, respectively. CONCLUSION: CT enhancement features of pulmonary metastasis in patients with primary cardiac angiosarcoma depend on the size of pulmonary nodules, with larger ones being heterogeneous and smaller ones homogeneous. Other signs are less noticed.

Comparison of fetal echocardiogram with fetal cardiac autopsy findings in fetuses with congenital heart disease.

OBJECTIVE: Although studies have compared fetal echo results with autopsy findings, investigations that compared multiple categories of congenital heart disease (CHD) are lacking. This study, therefore, aimed to compare fetal echocardiographic diagnoses with cardiac autopsy findings and evaluate the diagnostic accuracy of fetal echocardiography (FE). METHODS: One hundred seventy-one specimens from fetuses diagnosed with CHD were collected after termination of pregnancy, and fetal autopsies were performed. FE and autopsy diagnoses were compared and the degree of their correspondence was categorized as "complete agreement" (FE results were in accordance with autopsy findings), "minor discrepancies" (autopsies verified the main FE diagnoses but added and/or revised some minor information), or "discordance" (autopsy findings were different from the primary diagnoses of FE). RESULTS: The "complete agreement" group accounted for 87.1% (149/171) of the total specimens. In 11.7% (20/171) of cases, autopsies disclosed new deformities and/or revised some echo results (minor discrepancies group). Minor abnormalities were frequently embodied in small septal defects and vascular malformations. A rare malformation of common pulmonary vein atresia was confirmed by autopsy in two fetuses, but both were misdiagnosed by FE (discordance group). CONCLUSIONS: Fetal echocardiographic diagnoses were mostly consistent with autopsy findings. The diagnostic discrepancies mainly consisted of rare cases and minor abnormalities missed or misdiagnosed by FE. Autopsies may help confirm, modify, or add information to prenatal echo results. They may also help sonographers have a better understanding of the anatomic structures of CHD, especially for rare lesions, which could further improve the diagnostic accuracy and integrity of FE.

Primary cardiac synovial sarcoma: a clinicopathological, immunohistochemical, and molecular genetics study of five clinical cases.

BACKGROUND: Primary cardiac synovial sarcoma was an exceedingly rare tumor that less reported. The study investigated the clinicopathologic, immunohistochemical, and molecular features of primary cardiac synovial sarcoma. METHODS: A total of five cardiac synovial sarcoma cases were assessed and reviewed using H&E, immunohistochemical and fluorescence in situ hybridization staining methods. Clinicopathological data were retrospectively analyzed and followed up. RESULTS: The cases occurred in four males and one female ranging in age from 23 to 48 years (mean, 32 years). The tumors were grossly large and solid (7.4-13.7 cm; mean 8.6 cm). Microscopically, clinical cases were biphasic (n = 2) and monophasic (n = 3) types and were diffusely immunoreactive for EMA, vimentin, and BCL-2. All cases demonstrated SS18 rearrangement by fluorescence in situ hybridization staining. Clinically, three patients died within 1 year after surgery, while one patient had bone metastasis and still carried the disease. One last patient underwent a heart transplant and survived without evidence of the disease. CONCLUSION: Cardiac synovial sarcoma was an aggressive tumor whose differentiation may be a continuous and complex morphologic spectrum. SS18 rearrangement demonstration by fluorescence in situ hybridization was decisive in our study for differential diagnosis of cardiac synovial sarcoma and other tumors. Cardiac synovial sarcoma usually endured poor survival rates. Patients in advanced stages may undergo heart transplantation as a means of improving their survival rates.

Isolated subclavian or brachiocephalic arteries with tetralogy of Fallot, left retro-aortic brachiocephalic vein.

BACKGROUND: Isolated subclavian or brachiocephalic artery are uncommon aortic arch anomalies. Here we report the anatomy and histology of this disease. METHODS: Four cases of congenital isolated subclavian or brachiocephalic artery in fetuses are described. RESULTS: We identified one case of right aortic arch with isolated left subclavian artery associated with the tetralogy of Fallot, two cases of right aortic arch with isolated left brachiocephalic artery (one case with left retro-aortic brachiocephalic vein), and one case of left aortic arch with isolated right subclavian artery associated with coarctation of the aorta and cervical aortic arch. The proximal subclavian or brachiocephalic artery is arterial duct. CONCLUSION: Aortic arches with an isolated subclavian or brachiocephalic artery are often associated with the tetralogy of Fallot. It also can be associated with rare abnormalities such as left retro-aortic brachiocephalic vein or cervical aortic arch. Isolated LBA can be associated with microdeletion chromosome 22q11.

Clinical and Imaging Features of Primary Cardiac Angiosarcoma.

This study aims to explore computed tomography (CT) and magnetic resonance imaging (MRI) features of patients diagnosed with primary cardiac angiosarcoma. The study involved the analysis of 12 patients diagnosed with primary cardiac angiosarcoma who underwent non-contrast (8/12) or contrast-enhanced CT (10/12) or MRI (4/12). Imaging appearances, including the tumor location and adjacent infiltration, were analyzed. All 12 lesions were located in the right atrium with a broad base. Adjacent invasion including the tricuspid valve and right ventricle (2/12), inferior or superior vena cava (2/12), pericardium (10/12), and right coronary artery (7/12) was common. On unenhanced CT scans, tumors in two patients were homogeneous in density, whereas the others were inhomogeneous. Ten patients showed heterogeneous enhancement. The enhancement pattern showed no direct correlation with the differentiation degree of the tumor. Four lesions manifested as heterogeneous intensity, with hyperintense hemorrhage foci on both T1- and T2-weighted MRI. Three showed rim enhancement and one showed patchy enhancement. Primary cardiac angiosarcoma often involves the right side of the heart with infiltration of peripheral structures. CT features include typical inhomogeneous density on unenhanced scans and heterogeneous centripetal enhancement on enhanced scans. A cauliflower-like appearance on both T1- and T2-weighted MRI is common. The characteristic enhancement pattern of MRI remains to be determined.

Self-Powered Non-Contact Triboelectric Rotation Sensor with Interdigitated Film.

Rotation detection is widely applied in industries. The current commonly used rotation detection system adopts a split structure, which requires stringent installation requirements and is difficult to miniaturize. This paper proposes a single-piece self-powered non-contact sensor with an interdigital sensitive layer to detect the rotation of objects. The electric field generated between a polyurethane (PU) film and a polytetrafluoroethylene (PTFE) film is utilized for perceiving the rotation. The surface of the PU film is subjected to wet etching with sulfuric acid to increase the surface area and charge density. Through finite element analysis and experimental testing, the effects of the areas of the sensitive films as well as the horizontal and vertical distances between them on the output voltage are analyzed. Tests are performed on adjustable-speed motors, human arms, and robotic arms. The results show that the sensor can detect the speed, the transient process of rotation, and the swing angle. The proposed rotation sensor has broad application prospects in the fields of mechanical automation, robotics, and Internet of Things (IoT).

Prenatal diagnosis of Marfan syndrome by fetal echocardiography: A case report and review of cardiovascular manifestations.

Neonatal Marfan syndrome (nMFS), phenotypically and genotypically distinct from the classical syndrome, is rarely diagnosed prenatally, and the cardiovascular prognosis is poor. This case report described one fetus diagnosed with nMFS by fetal echocardiography. The main features were cardiomegaly, and atrioventricular valves prolapse with moderate regurgitation and dilated great vessels. Extracardiac malformations included right diaphragmatic eventration, bilateral pyelectasis, and lengthy femur. Pathological examination confirmed the findings of fetal echo, and all cardiac valves were dysplastic. Sanger sequencing revealed a deletion mutation affecting exon 30 of the fibrillin 1 (FBN1) gene. Echocardiography is essential for prenatal diagnosis, and multivalve dysplasia is common among those patients.

Comparison of prenatal ultrasound and postmortem findings in fetuses with common pulmonary vein atresia.

BACKGROUND: The previous literature on common pulmonary vein atresia (CPVA) mainly consists of neonatal case reports. There is a lack of research on the prenatal diagnosis of CPVA. METHODS: We conducted a retrospective study of all fetuses with CPVA confirmed by autopsy between August 2010 and May 2018. Prenatal echocardiographic features, autopsy findings, and genetic test results were analyzed. We compared fetal CPVA with total anomalous pulmonary venous return (TAPVR) and neonatal CPVA. RESULTS: During the study period, fetal echocardiography was performed on 31 617 fetuses. Six cases of CPVA were identified by autopsies, including 1 case performed with a cardiovascular cast. All 6 cases (100%) had asplenia syndrome (AS) and bilateral superior vena cava (BSVC). In 1 case (16.7%), the prenatal ultrasound results were in complete agreement with the postmortem findings. Four cases (66.7%) were misdiagnosed as TAPVR by prenatal echocardiography. For the remaining case (16.7%), no pulmonary venous anomalies were detected on prenatal echocardiography. No aneuploidy was identified in any of the cases. There were no statistically significant differences among the proportions of associated complex anomalies and AS between the fetal CPVA and TAPVR groups. The proportion of associated complex anomalies and AS in the fetal CPVA group was higher than that in the neonatal group (P < 0.05). CONCLUSIONS: Prenatal diagnosis of fetal CPVA is difficult and challenging even for experts. Our study showed that fetal CPVA is often combined with AS, complex cardiac malformations, and BSVC. These findings may help in the diagnosis of fetal CPVA.

Second-harmonic generation in a metal-clad nonlinear optical waveguide.

Efficient optical nonlinear effects in waveguides play an important role in integrated photonic functionalities. The dispersion characteristics need to be well designed to satisfy the phase-matching condition of the interacting waves in waveguides. Here we demonstrate a novel phase-matching process of second-harmonic generation (SHG) in a symmetrical metal-cladding optical waveguide (SMCOW) with a nonlinear guiding layer. Ultrahigh order modes in SMCOWs possess small propagation constants, and can be actively tuned to satisfy the phase-matching condition via free-space coupling. We establish a model of SHG in the SMCOW and experimentally verify it as well. This mechanism could also be applied to or referenced in other nonlinear frequency conversion processes.

[Autopsy findings of 19 cases of pulmonary vein abnormalities associated with fetal cardiac anomalies].

OBJECTIVE: To improve the diagnostic accuracy of fetal pulmonary venous abnormalities through the analysis of the fetal pulmonary vein anatomy. METHODS: 234 cases of congenital cardiac abnormalities were detected by echocardiography during pregnancy in An Zhen Hospital, Capital Medical University from May 2010 to August 2015. Autopsy was then performed. The type of fetal pulmonary venous malformation, cardiac abnormalities, systemic venous malformations, and other internal organs deformities were documented. RESULTS: There were ninteen cases of pulmonary venous malformations among the 234 cases of fetal congenital heart disease. These included two cases of congenital pulmonary venous hypoplasia (CPVH) or atresia, four cases of partial anomalous pulmonary venous drainage (PAPVD), seven cases of total anomalous pulmonary venous drainage (TAPVD), five cases of atresia of common pulmonary vein (CPV), one case of congenital pulmonary venous hypoplasia with total anomalous pulmonary venous drainage. There were eleven cases with single ventricle, eight cases with right aortic arch, seven cases with single atrium and six cases with pulmonary valve stenosis. Eleven cases had pulmonary hypoplasia and nine cases had abnormal spleen. CONCLUSIONS: There are many variations in pulmonary venous abnormalities associated with severe and complex cardiac abnormalities and internal organs malformation. Care should be exercised during autopsy examination to look for all branches of the pulmonary vein.

[Echocardiographic diagnoses of congenital atrial appendage aneurysm: from fetus to adult].

OBJECTIVE: To summarize the echocardiograhic features of atrial appendage aneurysm (AAA) in fetuses, children and adults and evaluate the value of echocardiography in the diagnosis of AAA. METHODS: The AAA echocardiographic features of 6 fetuses, 1 child and 4 adults were retrospectively analyzed. And the diagnoses were confirmed by autopsy, postnatal follow-ups, computed tomography (CT) and surgery. RESULTS: Left atrial appendage aneurysm was more common than right atrial appendage aneurysm. AAA had the same echocardiographic characteristics in fetuses, children and adults. And it was usually combined with other complex cardiac malformations in fetuses while single cardiac abnormality was predominant in children and adults. CONCLUSION: Fetuses, children and adults with AAA may be found by echocardiography in time so that echocardigraphy has an important role in diagnosing AAA.

[Clinicopathologic features of intravenous leiomyomatosis with intracardiac extension].

OBJECTIVE: To investigate clinicopathological features, immunophenotype and differential diagnosis of intravenous leiomyomatosis with intracardiac extension. METHODS: Clinical manifestations, morphologic features, and immunohistochemical staining were retrospectively analyzed in 19 cases of intravenous leiomyomatosis with intracardiac extension. RESULTS: The patients' age ranged from 33 to 59 years (mean 44 years). Clinical presentation included chest tightness, palpitation, dyspnea, edema of low extremity, abdominal distention or hypermenorrhea. However, a few patients were asymptomatic. Grossly, intravenous leiomyomatosis in most cases demonstrated coiled or nodular growth within the myometrium with worm-like involvement of the uterine vein in broad ligament or other pelvic veins with continued extension into the vena cava and the heart. The intravenous tumor surface was generally smooth and rubbery, with a greyish-white color. Microscopically, the tumors were composed of spindle cells with rare mitotic figures and the presence of abundant thick-walled vessels. Marked fibrosis, hyalinization, myxoid and edematous changes were common. The tumor cells were positive for SMA, ER, PR and desmin but negative for HMB45 and S-100. CD10 and CD34 were positive in 4 patients and negative in the remaining cases. CONCLUSION: Intravenous leiomyomatosis with intracardiac extension is a rare disease among women of child-bearing age, with specific morphologic manifestations and immunohistochemical profiles.

Primary cardiac paraganglioma with intratumoral coronary artery aneurysm.

We report here the surgical treatment of a rare case of primary cardiac paraganglioma with an intratumoral coronary artery aneurysm.

[Feasibility of using intravascular loopless monopole antenna to image atherosclerotic plaque in a porcine model with 3.0 T magnetic resonance imaging].

OBJECTIVE: To investigate the feasibility of using intravascular loopless monopole antenna (ILMA) to image atherosclerosis plaque in a porcine model with 3.0T magnetic resonance imaging (MRI). METHODS: Atherosclerosis model was established by feeding high fat diet combined with balloon catheter injury to the endothelium in 6 pigs. After 3 months, animals underwent MRI and ILMA examination. The ILMA was invasively inserted to the distal part of abdominal vein and bilateral common iliac veins. MR sequences including T1 weighted imaging (T1WI), T2WI were obtained. MR image data were transferred to post-processing station. Luminal border and external elastic membrane of the vessel were reconstructed based on the MR images. After co-register these images, vessel area, lumen area, vessel wall area and plaque burden in the same lesions imaged by different modality were calculated and compared. Finally, all animals were scarified and hematoxylin eosin (HE) staining was performed in the targeted vessels. Diagnostic accuracy of MR in delineating vessel wall and detecting plaque were analyzed and calculated by comparing with pathological results. RESULTS: The atherosclerotic model was successfully established in all 6 pigs. Good agreement of delineating vessel area, lumen area vessel, wall area and plaque burden were found between MRI and pathology with r value of 0.98, 0.95, and 0.96, respectively (P < 0.001). Compared with pathological findings, the plaque component in corresponding area imaged by MR was as follows: sensitivity and specificity of detecting lipid plaque were 77% and 69%, kappa value was 0.75 ± 0.19 (P < 0.01); sensitivity and specificity on detecting fibrotic plaque were 78% and 73%, Κ value was 0.78 ± 0.18 (P < 0.01). The sensitivity and specificity of detecting calcified plaque were 100%. ILMA results showed that the average lumen area was 49.72 mm(2), average vessel area was 124.08 mm(2), and the average vessel wall area was 74.37 mm(2), ILMA slightly overestimated these indexes as compared with pathological results. CONCLUSION: The results showed that ILMA could be used to image deepened artery and atherosclerotic plaque. Detected plaque size, vessel area, lumen area vessel, wall area, and plaque burden were comparable to pathological findings. It may thus provide an alternative method for detecting atherosclerotic plaque in future research work.

Detection of atherosclerotic plaque progression in the abdominal aorta of rabbits with 3T magnetic resonance imaging.

BACKGROUND: With features of high tissue contrast, MRI can be used for the qualitative and quantitative evaluation of atherosclerosis plaques. In this study we investigated the development of atherosclerosis plaque with high resolution 3T MRI in a rabbit model and compared the findings with the histopathological results. METHOD: Twenty male New Zealand white rabbits were randomly allocated into an experimental group (n = 16) and a control group (n = 4). Atherosclerotic lesions were induced in the abdominal aorta by balloon injury and cholesterol feeding. Multiple sequences MRI examination (ToF, T1WI, T2WI, and CE T1WI) were performed at the 2nd, 3rd, and 4th months after aortic denudation. Vessel wall thickness, total vessel area, lumen area, and vessel wall area were recorded. Plaque components were analyzed using histological results as a standard reference. RESULTS: Seventeen rabbits (14 in the experimental group and 3 in the control group) received all three MR examinations. Gradually, from 2 months to 4 months, vessel wall thickness and area in the experimental group increased significantly compared with the control group (P < 0.01). In the lumen area progressive stenosis was not found, even a slight dilation had developed in the experimental group. Lipid, fibrotic and calcified plaques can be differentiated by MR image. According to histological results, MRI had good performance in detection of lipid plaque. CONCLUSION: MRI can be used to monitor progression of atherosclerosis and differentiate plaque components.

In vivo MR imaging of plaque disruption and thrombus formation in an atherosclerotic rabbit model.

Our aim is to introduce an atherosclerotic rabbit model for inducing atherosclerosis lesions in rabbits, and to validate the model in vivo with 3T high resolution magnetic resonance imaging of the thrombosis followed a pharmacologically triggered plaque disruption. Twenty male New Zealand White rabbits were randomly allocated into an experimental group (n = 16) and a control group (n = 4). The aortic wall injuries were induced by an intravascular balloon in the experimental group rabbits after feeding them with a high cholesterol diet for 2 weeks. The pharmacological triggering with Russell's viper venom and histamine was performed after totally 16 weeks of intermittent cholesterol feeding. All of the animals underwent both the pre-trigger and post-trigger MR examinations including TOF, T1WI, T2WI and post contrast T1WI. Euthanasia was performed in all rabbits; gross anatomy and histological specimen of aorta were obtained. MR images were analyzed and compared with histological results. Compared with the control group rabbits, the aorta of the experimental group rabbits in the pre-triggered MR images showed an increased vessel wall thickening, luminal narrowing, and vessel wall enhancement. Fourteen rabbits survived the triggering, and 8 of them developed thrombosis (58.1%). No thrombus was found in the control group. The accuracy of the multi-sequences MR including TOF, T1WI, T2WI and post contrast T1WI was 87.1% (27/31) for detecting thrombus. MR data significantly correlated with the histopathology data for both thrombus length (r = 0.94, P < 0.01) and thrombus location (r = 0.85, P < 0.01), respectively. The study demonstrated that MR reliably determined the plaque disruption and thrombus formation in the atherosclerotic rabbit model.