CT features of malignant perivascular epithelioid cell carcinoma of the liver / 中华肝胆外科杂志

Objective:To study the CT manifestations of malignant hepatic perivascular epithelioid cell carcinoma (PEComa).Methods:Clinical data of nine patients undergoing surgery with histologically confirmed malignant hepatic PEComa in Yueqing People's Hospital, Wenzhou People's Hospital, and Yongjia County People's Hospital from January, 2010 to June, 2022 were retrospectively collected, including two males and seven females with a median age of 47 (43, 56) years old. The CT findings, including tumor size, shape, boundary, density, and enhancement patterns, were analyzed.Results:CT scans showed that all nine tumors were single lesions. Five tumors were located in right liver lobe, three in left lobe, and one in caudate lobe. The median tumor diameter was 5.7 (range, 3.3-16.0 ) cm. In terms of tumor shape, three were round, four were quasi-circular, and two were irregular. Eight tumors had well-defined boundaries, while one was ill-defined. Nine tumors showed uneven densities and were lower than the adjacent liver parenchyma. Four tumors had a false capsule, one contained fatty tissue, and six had necrotic and cystic changes. In the arterial phase of contrast-enhanced CT scanning, two tumors showed moderate enhancement and seven showed significant enhancement. In the portal venous and delayed phases, enhancement decreased significantly in four cases, showing a " fast-in and fast-out" pattern. In four cases, the enhancement persisted, showing a " fast-in and slow-out" pattern. One case showed no enhancement in any phase but had a strip-like enhanced vessel inside the tumor. Five cases had significantly thickened vessels around the tumor.Conclusion:The CT manifestations of PEComa are as follows: round or quasi-circular lesions with well-defined boundaries, uneven low densities, significant enhancement in the arterial phase and rapid washout or persistent enhancement in the portal venous and delayed phases.

Correlation between enlarged perivascular space and cerebral venous reflux in recent small subcortical infarcts within the lenticulostriate artery territory / 中华神经科杂志

Objective:To summarize the incidence of cerebral venous reflux (CVR) in patients with recent small subcortical infarct (RSSI) and explore its correlation with enlarged perivascular spaces (EPVS).Methods:Patients with RSSI in the lenticulostriate artery admitted to the Department of Neurology of the First Affiliated Hospital of Zhengzhou University from January 2019 to December 2022 were included. The baseline demographic data, medical history, and laboratory results of the patients were collected. CVR was assessed by time-of-flight magnetic resonance angiography. Patients were stratified into 2 groups based on the presence (CVR group) or absence of CVR (non-CVR group), and baseline characteristics as well as laboratory test results were compared between the 2 groups. The location and number of EPVS were evaluated using a visual grading scale, with EPVS with higher scores defined as high-grade EPVS (HEPVS). Simultaneous evaluation of cerebral white matter hyperintensities and lacunar infarctions was conducted, followed by intergroup comparisons. The relationship between EPVS and CVR was studied using multiple Logistic regression analysis.Results:A total of 571 patients with RSSI in the lentiform artery area were ultimately included, including 180 females (31.5%). Their age was (59.37±12.87) years. Among them, 73 patients (12.8%) exhibited CVR based on imaging findings, so the incidence of CVR was 12.8%. In comparison between the CVR group ( n=73) and the non-CVR group ( n=498), the proportion of females [21.9% (16/73) vs 32.9% (164/498), χ 2=3.578, P=0.059] was lower and the proportion of history of smoking [38.4% (28/73) vs 27.7% (138/498), χ 2=3.499, P=0.061] was higher in the CVR group, but without statistical significance. Additionally, the history of alcohol consumption [34.2% (25/73) vs 21.7% (108/498), χ 2=5.621, P=0.018] and the proportion of patients with concomitant HEPVS in the basal ganglia area [41.1% (30/73) vs 25.3% (126/498), χ 2=7.999, P=0.005] was higher in the CVR group with statistical significance. Multiple Logistic regression analysis showed that HEPVS in the basal ganglia region remained independently associated with CVR ( OR=1.988, 95% CI 1.190-3.320, P=0.009). Conclusion:EPVS in the basal ganglia region is significantly associated with CVR in the RSSI population, suggesting that venous dysfunction may be closely related to the formation of EPVS.

Clear cell myomelanocytic tumor of ligamentum teres

ABSTRACT Clear cell myomelanocytic tumor (CCMMT) of the falciform ligament/ligamentum teres is a rare hepatic tumor, a variant of the perivascular epithelioid cell tumor (PEComa) family. CCMMT is the rarest variant of hepatic PEComas. Only a few cases of CCMMT have been reported in the English literature. Because of its rarity, less is known about its biological behavior. We present a case of a 31-year-old female who complained of abdominal pain, bilious vomiting, and abdominal fullness over two months. The radiological impression was of focal nodular hyperplasia. The histological examination of the resection specimen revealed a well-circumscribed tumor arranged in fascicles, sheets, and a whorling pattern. The tumor cells were spindle to epithelioid shaped with abundant clear to pale eosinophilic cytoplasm. The tumor cells expressed both myoid (smooth muscle actin) and melanocytic (MelanA and HMB45) markers, while they were negative for hepatocytic and vascular markers. Thus, based on histology and immunohistochemistry, a diagnosis of CCMMT was made. This case presents the diagnostic challenges of CCMMT and discusses the differential diagnosis with a literature review.

Sistema glinfático: aspectos anatómicos, fisiológicos e implicaciones clínicas / Glymphatic system: Anatomo-physiological principles and their clinical implications

Introducción: El sistema glinfático comprende el conjunto de rutas perivasculares tanto arteriales como venosas que se encuentran en estrecha asociación con células astrogliales y que permiten la interacción entre el líquido cefalorraquídeo (LCR) y el líquido intersticial cerebral (LIC), para llevar a cabo procesos como la depuración de los metabolitos de desecho celular, o la distribución de nutrientes, así como contribuir al metabolismo cerebral local, la transmisión de volumen y la señalización paracrina cerebral. Contenidos: Este artículo busca profundizar en los conceptos anatómicos y fisiológicos, hasta el momento descritos, sobre este sistema macroscópico de transporte. Se realiza una búsqueda bibliográfica de revisiones y estudios experimentales sobre la anatomía, la fisiología y las implicaciones fisiopatológicas del sistema glinfático. Conclusiones: La identificación anatómica y funcional del sistema glinfático ha ampliado el conocimiento sobre la regulación del metabolismo cerebral en cuanto a distribución de nutrientes y cascadas de señalización celular. Al establecer una interacción entre el espacio subaracnoideo subyacente y el espacio intersticial cerebral, el sistema glinfático surge como uno de los mecanismos protagonistas de la homeostasis cerebral. La disfunción de esta vía hace parte de los mecanismos fisiopatológicos de múltiples trastornos neurológicos, ya sea por la acumulación de macromoléculas, como ocurre en la enfermedad de Alzheimer, o por la reducción del drenaje de sustancias químicas y citocinas proinflamatorias en patologías como la migraña o el trauma craneoencefálico.

Introduction: The glympathic system comprises the set of perivascular routes, arterials or venous, that are found in close relationship with astroglial cells and allow interaction between the cerebrospinal fluid (CSF) and the interstitial brain fluid (ISF), to carry processes like cell-wasting metabolites depuration, nutrients distribution, as well as make a contribution in the local brain metabolism, volumen transmition and brain paracrine signaling. Contents: This article seeks to deepen in the anatomical and physiological concepts, so far described, about this macroscopic transport system. A bibliographic search of reviews and experimental studies on the anatomy, physiology and pathophysiological implications of the glymphatic system is carried out. Conclusions: Anatomical and functional identification of glympathic system has broaden the knowledge about regulation of brain metabolism on the nutrients distribution and cell signaling cascades. When setting an interaction between the subarachnoid space and the brain interstitial space, the glymphatic system arise as one of the leading mechanisms of brain homeostasis. Disfunction of this pathway makes part of the patophysiological mechanisms of multiple neurological disease, either be by collection of macromolecules as in Alzheimer's disease, or by the reduction of inflammatory cytokines and chemical substances drainage as in migraine or traumatic brain injury (TBI).

Updated Understanding of the Glial-Vascular Unit in Central Nervous System Disorders / 神经科学通报·英文版

The concept of the glial-vascular unit (GVU) was raised recently to emphasize the close associations between brain cells and cerebral vessels, and their coordinated reactions to diverse neurological insults from a "glio-centric" view. GVU is a multicellular structure composed of glial cells, perivascular cells, and perivascular space. Each component is closely linked, collectively forming the GVU. The central roles of glial and perivascular cells and their multi-level interconnections in the GVU under normal conditions and in central nervous system (CNS) disorders have not been elucidated in detail. Here, we comprehensively review the intensive interactions between glial cells and perivascular cells in the niche of perivascular space, which take part in the modulation of cerebral blood flow and angiogenesis, formation of the blood-brain barrier, and clearance of neurotoxic wastes. Next, we discuss dysfunctions of the GVU in various neurological diseases, including ischemic stroke, spinal cord injury, Alzheimer's disease, and major depression disorder. In addition, we highlight the possible therapies targeting the GVU, which may have potential clinical applications.

Multi-slice spiral CT manifestations of perivascular epithelioid cell tumors / 中国基层医药

Objective:To investigate CT manifestations of perivascular epithelioid cell tumors.Methods:The multi-slice spiral CT manifestations in 11 patients with perivascular epithelioid cell tumors which were pathologically confirmed in the First Affiliated Hospital of Shantou University Medical College between October 2012 and August 2021 were retrospectively analyzed.Results:Perivascular epithelioid cell tumors were located in the kidney of eight patients, in the right lobe of the liver of two patients, and in the descending colon of one patient. The largest tumor, approximately 20.0 cm × 26.0 cm × 11.0 cm, was located in the kidney. The smallest tumor, 2.4 cm × 2.6 cm × 3.4 cm, was located in the colon. CT plain scans showed uniform density without bleeding, calcification, or fat in two patients, and non-uniform density with fatty change in two patients. Contrast-enhanced CT images showed non-uniform enhancement in the arterial phase in 10 patients and uniform enhancement in one patient. Contrast-enhanced CT images showed decreased degrees of enhancement of the lesions in 11 patients from the portal venous phase to the delayed phase and dilated and tortuous vascular shadows in 11 patients.Conclusion:The multi-slice spiral CT manifestations of perivascular epithelioid cell tumors are characteristic and the multi-slice spiral CT is of value for the diagnosis of perivascular epithelioid cell tumors.

Correlation between optic nerve tissue thickness and basal ganglia-enlarged perivascular space in patients with recent small subcortical infarct / 中华老年心脑血管病杂志

Objective To explore the relationship between optic nerve tissue thickness and enlarged perivascular space(EPVS)in patients with recent small subcortical infarct(RSSI).Methods A total of 72 RSSI patients admitted to Department of Neurology of Changzhou Second People's Hospital affiliated to Nanjing Medical University from November 2021 to December 2022 were re-cruited in this study.All patients underwent cranial MRI,optical coherence tomography(OCT)and fundus photography.The thickness of retinal nerve fiber layer(RNFL)and ganglion cell-inner plexiform layer(GCL-IPL)were measured by software semi-automatic segmentation,and fundus lesions such as fundus hemorrhage and exudation were observed through fundus photography.The severity of EPVS was graded by visual quantitative evaluation,and then the patients were divided into no to mild EPVS group(32 cases)and moderate to severe EPVS group(40 cases).Basal gan-glia EPVS(BG-EPVS)and semioval center EPVS(CSO-EPVS)were further graded and grouped into no to mild BG-EPVS group(31 cases)and moderate and severe BG-EPVS group(41 cases),and no to mild CSO-EPVS group(39 cases)and moderate and severe CSO-EPVS group(33 cases).The clinical data of each group were compared separately.Results There were statistical differences in age,ratio of hypertension and thicknesses of RNFL and GCL-IPL between the no to mild EPVS group and the moderate to severe EPVS group(P＜0.05,P＜0.01),between the no to mild BG-EPVS and the moderate to severe BG-EPVS group(P＜0.01).Multivariate logistic re-gression analysis showed that age(OR=1.207,95％CI:1.028-1.416,P=0.021),hypertension(OR=7.264,95％CI:1.079-11.929,P=0.042),RNFL thickness(OR=0.753,95％CI:0.617-0.915,P=0.004)and GCL-IPL thickness(OR=0.848,95％CI:0.758-0.949,P=0.004)were independent factors of BG-EPVS grade.Spearman correlation analysis indicated that the thicknes-ses of RNFL and GCL-IPL were negatively correlated with BG-EPVS grade(r=-0.571,r=-0.649,P＜0.01).Conclusion RNFL and GCL-IPL thicknesses are independent factors for BG-EPVS grade and negatively correlated with BG-EPVS grade.OCT has important clinical value in assessing the severity of EPVS lesions in RSSI patients.

Two cases report of perivascular epithelioid cell tumors of the kidney / 中华泌尿外科杂志

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor, mostly benign, and malignant is rare. In this paper, we report a 71-year-old male patient with left renal tumor and a 59-year-old patient with right renal tumor who underwent laparoscopic partial nephrectomy and laparoscopic radical total right nephrectomy, respectively. Postoperative pathology showed benign PEComa and malignant PEComa, respectively. Two years and 10 months of follow-up were given to the benign patient and the malignant patient after surgery, and both patients were in good general condition with no tumor recurrence or metastasis.

Recent advance in neutrophil extracellular traps in ischemic stroke / 中华神经医学杂志

Neutrophil extracellular traps (NETs) are compounds composed of depolymerized DNA fibers and antimicrobial peptides released by neutrophils. NETs formation not only plays a role in pathological process of non-infectious diseases such as cystic fibrosis, systemic lupus erythematosus, diabetes and cancer, but also is closely related to many central nervous system diseases. In recent years, a large number of studies have found the presence of neutrophils and NETs in perivascular space of the infarcted lesions in patients with ischemic stroke (IS) and corresponding animal models. This article provides a review on NETs formation and clearance process, characteristics of NETs changes after IS, pathological processes involved in NETs after IS, and effects of NETs on neurons, to provide some references for IS.

Research progress in the evaluation of glymphatic system function by the DTI-ALPS method / 中南大学学报(医学版)

The glymphatic system can remove metabolic wastes from the brain, which plays a significant role in maintaining the homeostasis of the central nervous system. It is an important basis for advanced cognitive functions such as learning and memory. Studies have analyzed the function of glymphatic system by diffusion tensor imaging analysis along the perivascular space (DTI-ALPS) recently. Compared with other invasive examinations that require fluorescent tracer technique or the injection of contrast agents, DTI-ALPS can evaluate the hydromechanics of the glymphatic system via quantifying the diffusion rate of water molecules in different directions, which turns out to be a non-invasive in vivo neuroimaging method. The ALPS-index calculated by the DTI-ALPS method is significantly correlated with the cognitive function in diseases of central nervous system and other system and can reflect the dynamic changes of diseases. In general, ALPS-index is expected to become a novel neuroimaging biomarker for predicting prognosis and clinical effects.

Qualitative and quantitative characteristics of the passive compartment underneath the myocardial bridge comprising the blood vessel adventitia and the perivascular connective tissue / Características cualitativas y cuantitativas del compartimento pasivo debajo del puente miocárdico que comprende la adventicia del vaso sanguíneo y el tejido conectivo perivascular

SUMMARY: Myocardial bridges are inborn anomalies frequently found in authopsies. Although tipically clinically silent, they are occasionally associated with severe clinical manifestations, e.g. myocardial ischemia or even sudden death. The pathophysiology and risk factors for these manifestations have not yet been completely elucidated. The connective tissue underneath the bridge has been considered as one of the factors the symptoms depend on. Thus, the aim of this research was to determine the histological characteristics of the connective tissue lying underneath the myocardial bridge and to contribute to a better understanding of the protective effects this passive compartment might have in prevention of severe clinical manifestations of myocardial bridging. The study was carried out on twenty hearts with myocardial bridges. Length of the bridge was determined using a precise electronic caliper. Sections of the myocardial bridges with the underlying connective tissue were obtained and prepared for qualitative and quantitative analysis. The connective tissue underneath the bridges was composed of adipose tissue and loose connective tissue in different ratios. The tissue underneath thin bridges was predominantly composed of adipose tissue, while loose connective tissue was the dominant component under thick bridges. The myocardial bridges had an average thickness of 0,98 ± 0.44 mm and an average length of 15,25±5,65 mm. We found a strong positive correlation between the myocardial bridge thickness and length (r = 0,860, p = 0,0001). The thickness of the passive connective tissue compartment under the myocardial bridges was 0,58±0,22 mm, and there was no correlation between this parameter and the myocardial bridge thickness (r = -0,011; p = 0,963). In the clinical evaluation of patients with these anomalies it is necessary to take into account independently the myocardial bridge thickness and length on one side and the thickness of the connective tissue lying underneath it on the other.

Los puentes miocárdicos son anomalías congénitas que se encuentran con frecuencia en las autopsias. Aunque típicamente éstos son clínicamente silenciosos, ocasionalmente se asocian con manifestaciones clínicas graves, como isquemia miocárdica o incluso muerte súbita. La fisiopatología y los factores de riesgo de estas manifestaciones aún no se han dilucidado por completo. El tejido conectivo debajo del puente se ha considerado como uno de los factores de los que dependen los síntomas. Por lo tanto, el objetivo de esta investigación fue determinar las características histológicas del tejido conectivo que se encuentra debajo del puente miocárdico y contribuir a una mejor comprensión de los efectos protectores que este compartimento pasivo podría tener en la prevención de manifestaciones clínicas graves de puente miocárdico. El estudio se llevó a cabo en veinte corazones con puentes miocárdicos. La longitud del puente se determinó utilizando un calibrador electrónico preciso. Se obtuvieron secciones de los puentes miocárdicos con el tejido conjuntivo subyacente y se prepararon para análisis cualitativo y cuantitativo. El tejido conectivo debajo de los puentes estaba compuesto de tejido adiposo y tejido conectivo laxo en diferentes proporciones. El tejido debajo de los puentes delgados estaba predominantemente compuesto de tejido adiposo, mientras que el tejido conectivo laxo era el componente dominante debajo de los puentes gruesos. Los puentes de miocardio tenían un espesor promedio de 0,98 ± 0,44 mm y una longitud promedio de 15,25 ± 5,65 mm. Encontramos una fuerte correlación positiva entre el grosor y la longitud del puente miocárdico (r = 0,860, p = 0,0001). El grosor del compartimiento de tejido conectivo pasivo debajo de los puentes miocárdicos era de 0,58±0,22 mm, y no hubo correlación entre este parámetro y el grosor del puente miocárdico (r = -0,011; p = 0,963). En la evaluación clínica de pacientes con estas anomalías es necesario tener en consideración de forma independiente el grosor y la longitud del puente de miocardio por un lado y el grosor del tejido conectivo que se encuentra debajo del mismo por el otro.

Study on imaging features of asymptomatic cerebral small vessel disease and related factors in the elderly population / 中华老年医学杂志

Objective:To investigate the incidence, neuroimaging features, and related factors for asymptomatic cerebral small vessel disease(CSVD)in the elderly population.Methods:A total of 201 elderly people with no neurological disease history who had undergone brain magnetic resonance imaging(MRI)examination from October 2019 to August 2020 were enrolled.We calculated the total CSVD score for each participant based on lacunar infarcts(LIs), white matter hyperintensities(WMH), enlarged perivascular spaces(EPVS), and cerebral microbleeds(CMBs)(0-4 points).CSVD neuroimaging features and the correlation between CSVD markers and clinical variables were analyzed.Results:In this study, 133 cases(66.2%)showed MRI features consistent with CSVD.Of whom, LIs were present in 44(21.9%), high-grade PVWMH in 88(43.8%), high-grade DWMH in 30(14.9%), basal ganglia EPVS in 61(30.3%), and CMBs in 92(45.8%).Total CSVD burden score( OR=1.876, 95% CI: 1.045-3.364, χ2=4.441, P=0.035), PVWMH( OR=2.821, 95% CI: 1.517-5.244, χ2=10.752, P=0.001), DWMH( OR=2.130, 95% CI: 1.108-4.092, χ2=5.145, P=0.023), and EPVS( OR=3.258, 95% CI: 1.675-6.334, χ2=12.129, P=0.000)were associated with hypertension.Total CSVD burden score, PVWMH, DWMH, EPVS, and CMB were correlated with increasing age( P<0.05).LIs was positively correlated with PVWMH( b=0.231, P=0.001), DWMH( b=0.247, P=0.000)and EPVS( b=0.215, P=0.001).There was a positive relationship between PVWMH and DWMH( b=0.546, P=0.000)as well as EPVS( b=0.388, P=0.000).DWMH was also positively correlated with EPVS( b=0.357, P=0.000)and CMB( b=0.177, P=0.009). Conclusions:The incidence of asymptomatic CSVD is high in the elderly population.The total CSVD score is a useful measure to evaluate asymptomatic cerebral small vessel disease in the elderly population.Neuroimaging features of asymptomatic CSVD are mainly correlated with age and hypertension.

CT, magnetic resonance imaging and clinicopathological characteristics of perivascular epithelioid cell tumor in children / 中华实用儿科临床杂志

Objective:To investigate the CT, magnetic resonance imaging(MRI) and clinicopathological cha-racteristics of perivascular epithelioid cell tumors (PEComas) in children, and improve the diagnostic accuracy of PEComas.Methods:The CT, MRI and clinicopathological characteristics of 6 children confirmed with PEComas by histopathology caming from Beijing Children′s Hospital, Capital Medical University during March 2019 and April 2022 were retrospectively analyzed.Results:The patients aged from 1.7 to 11.9 years old (with mean age of 7.6 years old). The male to female ratio was 1∶2.Of the 6 solitary tumors identified, 2 cases were found in the kidney, 2 cases in the abdominal cavity, 1 case in the pelvic cavity, and 1 case in the groin.The solitary tumors had well-defined borders.Five solitary tumors were shaped like an ellipse and 1 solitary tumor was multinodular-shaped.Lace-like or nodular changes were observed at tumor margins(3 cases). The maximum diameters of the tumors ranged from 4.8 to 15.9 cm (mean value: 9.3 cm). Five tumors showed peripheral invasion and 2 cases had lymph node metastasis.Two patients were complicated with tuberous sclerosis.One case suffered from tumor recurrence after operation.The tumors (6 cases) were isodensity or low density on the plain CT scan, and gradual heterogeneous enhancement was observed after injection of the contrast agent.A majority of the tumors had tortuous (5 cases) and thickened blood vessels inside, and a few of them (2 cases) underwent gravel-like calcification.Patch-like or nodule-like high signal intensity could be seen in the center of the tumors (3 cases) on MRI T1WI and T2WI, while the peripheral part of the lesions showed iso-signal intensity (equal to muscle). On diffusion weighted imaging(DWI) (b=800 s/mm 2), the lesions exhibited restricted diffusion at the peripheral part with significant enhancement, and iso-signal intensity at the central part with none or slight enhancement.Histological classification results revealed that 1 of 6 cases was a benign PEComa and 5 cases were malignant PEComas.The expression rates of Melan-A and SMA were 100.0% and 83.3%, respectively. Conclusions:The CT, MRI and pathological manifestations of PEComas in children have some distinct features.PEComas are often an oval-like mass with clear borders and nodular margins in the abdominal and pelvic cavity, and tortuous and thickened blood vessels can be seen in the tumor.The periphery of the tumor is the main region to be enhanced by the contrast agent.Local invasion and distant metastasis can be observed.Most of the tumors are malignant in pathology.Both melanocyte and muscle cell markers can be found in the expression of immunohistochemistry.

Clinical diagnosis and treatment of liver perivascular epithelioid cell neoplasms / 中华肝胆外科杂志

Objective:To analyze the clinical features and prognosis of liver perivascular epithelioid cell neoplasms (PEComa).Methods:The clinical data of 12 patients with liver PEComa diagnosed by pathology at the First Affiliated Hospital of Xi 'an Jiaotong University from October 2011 to November 2021 were retrospectively analyzed. There were 1 male and 11 females, with a median age of 44 (range 20 to 63) years old. The clinical manifestations, laboratory examinations, imaging features, treatment methods, postoperative pathological features and treatment outcomes of these patients were collected and analysed. Postoperative follow-up by telephone or patient's follow-up records.Results:Among the 12 patients with hepatic PEComa, 8 patients (66.7%) were asymptomatic and 11 patients (91.7%) had a single lesion. All patients underwent surgical treatment, including local tumor resection in 10 patients (83.3%) and extended hemihepatectomy in 2 patients (16.7%). Enhanced CT showed the lesion to be a quasi-round homogeneous low-density mass, enhanced in arterial phase with hepatic artery branches in the lesion, and decrease in enhancement degrees in portal vein phase and delayed phase. Postoperative pathology of the lesions in all the 12 patients was benign. Immunohistochemical results showed that the positive rates of melanoma cell markers HMB45, Melan-A and smooth muscle actin were 100.0%(12/12), 83.3%(10/12) and 91.7%(11/12) respectively. The median follow-up period was 27 months, and no recurrence or metastasis was found.Conclusion:Hepatic PEComa occurred commonly in women with obscure symptoms. The lesion was mainly single and it had no correlation with hepatitis. It is easily confused with primary liver cancer and liver metastasis on medical imagings. PEComa expressed markers of both melanocyte and smooth muscle cell, and radical surgical resection resulted in good results.

Relationship between perivascular space enlargement and cognitive and motor longitudinal changes in patients with Parkinson′s disease / 中华神经科杂志

Objective:To investigate the correlation between basal ganglia (BG) enlarged perivascular space (EPVS; BG-EPVS) and cognitive and motor longitudinal changes in patients with newly diagnosed Parkinson′s disease and its different motor subtypes [tremor dominant (TD), postural instability and gait disorder (PIGD)].Methods:A total of 131 Parkinson′s disease patients from the Parkinson Progression Markers Initiative (PPMI) database were screened and their clinical data were collected at baseline, 1 year and 2 years of follow-up. The number of EPVS in different brain regions was assessed on axial T 2-weighted images by cranial imaging data, and they were divided into two groups according to the degree of EPVS: BG-EPVS- and BG-EPVS+. Parkinson′s disease patients were divided into TD and PIGD groups by Movement Disorder Society Unified Parkinson′s Disease Rating Scale (MDS-UPDRS) score, and the number and clinical data of EPVS were compared between the two groups, and the correlation between the number and degree of BG-EPVS at baseline and longitudinal changes in clinical outcome measures of Parkinson′s disease and its different motor subtypes (TD, PIGD) was analyzed. Results:BG-EPVS was positively correlated with age ( r=0.32, P<0.01), Hoehn & Yahr stage ( r=0.21, P<0.05), serum neurofilament light chain ( r=0.18, P<0.05) and Epworth Sleepiness Scale score ( r=0.20, P<0.05) in all Parkinson′s disease patients. At baseline and 2 years, the number of BG-EPVS was more in the PIGD group than in the TD group (11.0±4.2 vs 9.0±3.8, t=2.18, P=0.03; 16.3±6.7 vs 12.6±4.6 , t=2.71 , P=0.007;after correction).At baseline, more BG-EPVS in patients with Parkinson′s disease and its motor subtypes (TD, PIGD) was significantly associated with baseline motor outcomes ( β=0.66, P=0.01; β=0.64, P=0.008; β=0.91, P=0.009), but not with cognitive outcomes. By linear mixed effects model analysis, BG-EPVS numbers and moderate to severe BG-EPVS were positively correlated with motor outcomes over time in patients with Parkinson′s disease and its motor subtypes (TD, PIGD) ( β=0.51, P=0.008; β=0.59, P=0.025; β=0.80, P=0.038). After dividing BG-EPVS in Parkinson′s disease patients into different degrees, moderate to severe BG-EPVS was positively correlated with motor outcomes over time ( β=3.30, P=0.031). Conclusion:In this longitudinal study, bigger baseline BG-EPVS numbers were found to be positively associated with longitudinal changes in dyskinesia severity in Parkinson′s disease patients, not with cognitive changes, and be able to predict decline in motor function over a 2-year follow-up period.

Dysfunction of lymphatic system and early Parkinson′s disease / 中华神经科杂志

Lymphatic system is the transportation way of cerebrospinal fluid and brain interstitial fluid exchange. And this system is a central nervous drainage system which plays an important role in drainaging and discharging of metabolic waste in the brain. The function of this system can be evaluated indirectly by the perivascular space on magnetic resonance imaging. Parkinson′s disease is a common neurodegenerative disorder. It may be helpful to control the progression of the disease if the changes of perivascular space can be dynamically observed in the early or even prodromal stage of the disease. This article reviews the relationship between lymphatic system disfunction and early stage of Parkinson′s disease.

Glymphatic system in Parkinson′s disease / 中华神经科杂志

Parkinson′s disease is a degenerative disease of the central nervous system with abnormal protein deposition in the brain as the main pathological changes. The onset of Parkinson′s disease is related to abnormal deposition protein removal disorders, and the relevant mechanisms are still unclear. The glymphatic system is a metabolic waste and abnormal protein deposition removal system in the brain. In recent years, studies have shown that the changes of aquaporin 4, perivascular space and dural lymphatic vessels in Parkinson′s disease are closely related to the level of relevant pathological proteins in cerebrospinal fluid, leading to the occurrence and development of Parkinson′s disease. The researches on structure and biomarkers of the glymphatic system in Parkinson′s disease in recent years are reviewed in this article.

Correlation of enlarged perivascular spaces with post-stroke cognitive impairment in patients with acute mild stroke / 中华神经医学杂志

Objective:To investigate the relations between enlarged perivascular spaces (EPVS) and cognitive impairment in patients with acute mild ischemic stroke.Methods:A total of 234 patients with acute mild ischemic stroke admitted to our hospital from October 2019 to June 2021 were chosen in our study. According to the Montreal Cognitive Assessment (MoCA) scores 7 d after admission, these patients were divided into normal cognitive function group (MoCA scores≥26) and cognitive impairment group (MoCA scores<26). The clinical data and imaging data of patients from the 2 groups were compared. Multivariate Logistic regression analysis was used to determine the independent influencing factors for post-stroke cognitive impairment (PSCI). Spearman correlation analysis was performed to analyze the correlations of severity degrees of EPVS of basal ganglia with MoCA total scores and each cognitive domain scores in patients from cognitive impairment group.Results:Among the 234 patients, 73 (31.2%) had normal cognitive function and 161 (68.8%) had cognitive impairment. As compared with normal cognitive function group, patients from cognitive impairment group had significantly older age, significantly less years of education, statistically higher fasting blood glucose level, significantly higher proportion of patients with moderate and severe basal ganglia EPVS, and significantly higher proportion of patients with white matter lesion (WML) grading 2 and 3 ( P<0.05). Multivariate Logistic regression analysis showed that age, years of education, basal ganglia EPVS and WML grading were independent influencing factors for PSCI ( OR=1.049, 95%CI: 1.007-1.093, P=0.021; OR=0.910, 95%CI: 0.832-0.995, P=0.039; OR=0.760, 95%CI: 1.176-2.637, P=0.006; OR=2.270, 95%CI: 1.219-4.228, P=0.010). Correlation analysis showed that the severity degrees of basal ganglia EPVS were negatively correlated with MoCA scores, and scores of visual space and executive ability scale, attention scale, language scale and delayed recall scale ( P<0.05). Conclusion:Acute mild ischemic stroke patients with older age, less years of education, severer basal ganglia EPVS and higher WML grading trends to have cognitive impairment; basal ganglia EPVS mainly affects the cognitive domains of visual space and executive ability, attention, language, and delayed recall.

Correlation between basal ganglia-enlarged perivascular space and executive function in patients with recent small subcortical infarct / 中华神经医学杂志

Objective:To identify the influencing factors for cognitive impairment in patients with recent small subcortical infarct (RSSI), and explore the relationship between enlarged perivascular space (EPVS) and executive function in patients with RSSI.Methods:From February to December 2021, 115 patients with RSSI accepted treatment in Department of Neurology, Changzhou Second People's Hospital Affiliated to Nanjing Medical University were enrolled. According to Montreal cognitive assessment (MoCA) scores, these patients were divided into normal cognitive function group (MoCA scores≥26, n=45) and cognitive impairment group (MoCA scores<26, n=70); univariate analysis was used to analyze the differences of general clinical data and EPVS volume between the two groups; multivariate Logistic regression analysis was used to identify the independent influencing factors for RSSI combined with cognitive impairment. Patients were further divided into EPVS non-mild group and EPVS moderate-severe group according to EPVS visual assessment; the differences of scores of different executive function domains were compared between the two groups; Spearman correlation analysis was used to observe the relationships of EPVS grading and volume with executive function. Results:Patients in the cognitive impairment group had significantly older age, significantly higher serum creatinine level, proportion of patients with moderate-severe basal ganglia EPVS (BG-EPVS) and BG-EPVS volume, and significantly lower years of education as compared with those in the normal cognitive function group ( P<0.05). Multivariate Logistic regression analysis showed that BG-EPVS volume ( OR=1.421, 95%CI: 1.028-1.965, P=0.034) was an independent risk factor for RSSI combined with cognitive impairment. MoCA total scores, scores of visual space and executive function domains in patients of the BG-EPVS moderate-severe group were significantly lower than those in patients of the BG-EPVS non-mild group, while Z-scores of Stroop color word test (SCWT) and trail making test (TMT), and total Z-scores of executive function were significantly higher than those in patients of the BG-EPVS non-mild group ( P<0.05). Spearman correlation analysis showed that BG-EPVS grading and volume were positively correlated with total Z-scores of executive function in RSSI patients with cognitive impairment ( r=0.439, P=0.001; r=0.410, P=0.001). Conclusion:BG-EPVS volume is an independent risk factor for cognitive impairment, and both BG-EPVS grading and volume are correlated with impairment degrees of executive function in cognitive function in RSSI patients.

Study of mechanism of bone marrow perivascular niche injured by 5-fluorouracil / 中国药理学通报

Aim To investigate the injury of 5-fluorouracil(5-FU)to perivascular hematopoietic niche via isolating mouse bone marrow perivascular mesenchymal progenitor cells in vitro and its related mechanism. Methods The perivascular mesenchymal progenitor cells were isolated from femurs and tibias of C57BL/6J mice with type Ⅱ collagenase and cultured in vitro. Agarose gel electrophoresis was used to detect specific niche genes expression. The viable cells were counted by Trypan blue; the cellular proliferation was detected by CCK-8; the apoptosis was detected by Annexin V/PI double staining, and the cell senescence was detected by β-galactosidase staining. The levels of malondialdehyde(MDA)and superoxide dismutase(SOD)were detected by enzymatic assay. Osteogenic and adipogenic differentiation potential of cells were detected by osteogenic and adipogenic differentiation experiment and osteogenic related genes qRT-PCR assay. The mRNA expression of hematopoietic growth factors was detected by qRT-PCR. Hematopoietic cells were co-cultured with perivascular mesenchymal progenitor cells, and the adhesion molecules and signal molecules between stromal cells and hematopoietic cells were detected, also hematopoietic cell activity, redox indicators and β-galactosidase specific cell senescence were detected. Results 5-FU caused simultaneous apoptosis and senescence of perivascular mesenchymal progenitor cells, inhibited cell proliferation, induced oxidative stress, led to osteogenic/adipogenic differentiation imbalance, and down-regulated the transcription of hematopoietic factors SCF, CXCL12, and G-CSF. For the interaction between stromal cells and hematopoietic cells, the binding effects of VLA-4/VCAM-1, ICAM-1/LFA1 were weakened and TPO/MPL and ANG-1/Tie-2 signals were impaired, leading to oxidative stress of hematopoietic cells and cell senescence. Conclusions 5-FU induces oxidative damage of perivascular mesenchymal progenitor cells and indirectly induces premature senescence of hematopoietic cells.