[Respiratory virus infection and its influence on outcome in children with septic shock].

Objective: To investigate respiratory virus infection in children with septic shock in pediatric care units (PICU) in China and its influence on clinical outcomes. Methods: The clinical data of children with septic shock in children's PICU from January 2018 to December 2019 in 10 Chinese hospitals were retrospectively collected. They were divided into the pre-COVID-19 and post-COVID-19 groups according to the onset of disease, and the characteristics and composition of respiratory virus in the 2 groups were compared. Matching age, malignant underlying diseases, bacteria, fungi and other viruses, a new database was generated using 1∶1 propensity score matching method. The children were divided into the respiratory virus group and non-respiratory virus group according to the presence or absence of respiratory virus infection; their clinical characteristics, diagnosis, and treatment were compared by t-test, rank sum test and Chi-square test. The correlation between respiratory virus infection and the clinical outcomes was analyzed by logistic regression. Results: A total of 1 247 children with septic shock were included in the study, of them 748 were male; the age was 37 (11, 105) months. In the pre-and post-COVID-19 groups, there were 530 and 717 cases of septic shock, respectively; the positive rate of respiratory virus was 14.9% (79 cases) and 9.8% (70 cases); the seasonal distribution of septic shock was 28.9% (153/530) and 25.9% (185/717) in autumn, and 30.3% (161/530) and 28.3% (203/717) in winter, respectively, and the corresponding positive rates of respiratory viruses were 19.6% (30/153) and 15.7% (29/185) in autumn, and 21.1% (34/161) and 15.3% (31/203) in winter, respectively. The positive rates of influenza virus and adenovirus in the post-COVID-19 group were lower than those in the pre-COVID-19 group (2.1% (15/717) vs. 7.5% (40/530), and 0.7% (5/717) vs. 3.2% (17/530), χ2=21.51 and 11.08, respectively; all P<0.05). Rhinovirus virus were higher than those in the pre-Covid-19 group (1.7% (12/717) vs. 0.2% (1/530), χ2=6.51, P=0.011). After propensity score matching, there were 147 cases in both the respiratory virus group and the non-respiratory virus group. Rate of respiratory failure, acute respiratory distress, rate of disseminated coagulation dysfunction, and immunoglobulin usage of the respiratory virus group were higher than those of non-respiratory virus group (77.6% (114/147) vs. 59.2% (87/147), 17.7% (26/147) vs. 4.1% (6/147), 15.6% (25/147) vs. 4.1% (7/147), and 35.4% (52/147) vs. 21.4% (32/147); χ2=11.07, 14.02, 11.06 and 6.67, all P<0.05); and PICU hospitalization of the former was longer than that of the later (7 (3, 16) vs. 3 (1, 7)d, Z=5.01, P<0.001). Univariate logistic regression analysis showed that the presence of respiratory viral infection was associated with respiratory failure, disseminated coagulation dysfunction, the use of mechanical ventilation, and the use of immunoglobulin and anti-respiratory viral drugs (OR=2.42, 0.22, 0.25, 0.56 and 1.12, all P<0.05). Conclusions: The composition of respiratory virus infection in children with septic shock is different between pre and post-COVID-19. Respiratory viral infection is associated with organ dysfunction in children with septic shock. Decreasing respiratory viral infection through respiratory protection may improve the clinical outcome of these children.

[Expression of programmed cell death ligand 1 and clinicopathological and immunological characteristics in fumarate hydratase-deficient renal cell carcinoma].

Objective: To investigate the expression of programmed cell death ligand 1 (PD-L1), clinicopathologic features, immunohistochemical expression and molecular characteristics in fumarate hydratase (FH)-deficient renal cell carcinoma and to explore the potential application of immunotherapy in the patients. Methods: There were six patients with FH-deficient renal cell carcinoma treated at the First Affiliated Hospital of Fujian Medical University between January 2020 and October 2022. The clinical data, histological morphology, immunophenotype, PD-L1 expression and next-generation sequencing results were tabulated and analyzed. Results: There were 6 patients, all male, age ranged from 37 to 72 years (mean 45.7 years). Four cases were high-grade (WHO/ISUP grade3-4) with 2 or more histologic patterns, including papillary (most common), glandular, tubular, vesicular, ethmoid, nest-like, cystic and solid structures. Two cases were low-grade which showed nest-like, glandular, or tubular arrangement with eosinophilic flocculent cytoplasm and small intracellular vacuoles. Immunohistochemical analysis revealed strong expression of 2SC in all 6 cases, negative expression of FH in 5 cases, and positive expression of GATA3 in 5 cases. In high-grade cases, the mean values of CD4 and CD8 positive T-lymphocytes in advanced tumor invasion were 180.3/mm2 and 130.5/mm2, respectively. PD-L1 combined positive scores (CPS) were 20, 50, 5 and 30, respectively. The Ki-67 proliferative index were 20%, 20%, 10% and 30%, respectively. In low-grade cases, the mean values of CD4 and CD8 positive T-lymphocytes were 123.0/mm2 and 100.5/mm2, respectively. The PD-L1 CPS score was 1, and the Ki-67 proliferation index was 3%. High-throughput sequencing showed FH gene somatic mutation in 3 cases, FH gene germline mutation in 2 cases, and FH gene mutation was not detected in one case. Conclusion: FH-deficient renal cell carcinoma is more commonly high-grade than low grade. FH and 2SC are immunohistochemical markers used in the diagnosis of FH-deficient renal cell carcinoma, and GATA3 positivity is supportive of the diagnosis. The tumor infiltration of high-grade FH-deficient renal cell carcinoma shows an increase in CD4 and CD8 positive T-lymphocytes, and high expression of PD-L1; thus, anti-PD-L1 immunotherapy can be used as a treatment option.

[Summary of experience with patterning cropped and shaped mesh repair for perineal hernia after abdominoperineal excision in rectal cancer].

Objective: To examine the patterning cropped and shaped mesh repair for perineal hernia after abdominoperineal excision (APE) in rectal cancer. Methods: The clinical data of 8 patients with perineal hernia after APE who accepted surgical treatment in the Department of Hepatopancreatobiliary and Hernia Surgery, the First Affiliated Hospital of Fujian Medical University from March 2017 to December 2022 were retrospectively reviewed. There were 3 males and 5 females, aged (67.6±7.2) years (range: 56 to 76 years). Eight patients developed a perineal mass at (11.3±2.9) months (range: 5 to 13 months) after APE. After surgical separation of adhesion and exposing the pelvic floor defect, a 15 cm×20 cm anti-adhesion mesh was fashioned as a three-dimensional pocket shape to fit the pelvic defect, then fixed to the promontory or sacrum and sutured to the pelvic sidewalls and the anterior peritoneum, while two side slender slings were tailored in front of the mesh and fixed on the pectineal ligament. Results: The repair of their perineal hernias went well, with an operating time of (240.6±48.8) minutes (range: 155 to 300 minutes). Five patients underwent laparotomy, 3 patients tried laparoscopic surgery first and then transferred to laparotomy combined with the perineal approach. Intraoperative bowel injury was observed in 3 patients. All patients did not have an intestinal fistula, bleeding occurred. No reoperation was performed and their preoperative symptoms improved significantly. The postoperative hospital stay was (13.5±2.9) days (range: 7 to 17 days) and two patients had postoperative ileus, which improved after conservative treatment. Two patients had a postoperative perineal hernia sac effusion, one of them underwent placement of a tube to puncture the hernia sac effusion due to infection, and continued irrigation and drainage. The postoperative follow-up was (34.8±14.0) months (range: 13 to 48 months), and 1 patient developed recurrence in the seventh postoperative month, no further surgery was performed. Conclusions: Surgical repair of the perineal hernia after APE can be preferred transabdominal approach, routine application of laparoscopy is not recommended, combined abdominoperineal approach can be considered if necessary. The perineal hernia after APE can be repaired safely and effectively using the described technique of patterning cropped and shaped mesh repair.

[Characteristics of daytime napping and its correlation with chronic diseases in Chinese adults].

Objective: To describe the prevalence of daytime nap habit in participants of the China Kadoorie Biobank (CKB) study, across 10 study regions and explore its correlation with prevalence of major chronic diseases. Methods: Participants with a self-reported pre-diagnosis of any cancer at baseline survey were excluded. Logistic regression models were used to analyze the differences in study regions and age distribution of the prevalence daytime nap habit, and its correlation with the prevalence of diabetes, hypertension, coronary heart disease (CHD), stroke, chronic obstructive pulmonary disease (COPD), and chronic liver diseases. Results: Among 510 145 participants, 39.9% had daytime nap habit in summer and 20.8% had daytime nap habit all the year round. Urban-rural differences were observed in the prevalence of summer nap habit and perennial nap habit. Daytime nap in summer was common in rural areas and Suzhou, with prevalence ranged from 32.9% to 73.3%. Haikou and Liuzhou had higher prevalence of perennial nap (60.4% and 63.3%). The proportion of people with daytime nap habit all the year round increased with age (P for trend <0.001), the proportion was highest in those aged 70- years (31.9%). Daytime nap habit in summer was positively correlated with the prevalence of diabetes, hypertension, CHD and chronic liver disease with OR of 1.10 (95%CI: 1.07-1.14), 1.03 (95%CI:1.02-1.05), 1.07 (95%CI: 1.02-1.12) and 1.07 (95%CI:1.00-1.14), respectively. Daytime nap habit all the year round was positively correlated with the prevalence of diabetes, hypertension, CHD, stroke, COPD and chronic liver disease with OR of 1.33 (95%CI: 1.29-1.37), 1.11 (95%CI: 1.09-1.13), 1.39 (95%CI: 1.33-1.45), 1.33 (95%CI: 1.26-1.41), 1.12 (95%CI: 1.08-1.16) and 1.27 (95%CI:1.18-1.37) respectively. Conclusion: There were regional and age differences in prevalence of daytime nap habit among CKB participants. Daytime nap habit, especially daytime nap habit all the year round, was positively correlated with the prevalence of major chronic diseases.

[Spicy food consumption and risk of lip, oral cavity and pharynx cancers: a prospective cohort study of Chinese adults].

Objective: To explore the association of spicy food consumption and risk of lip, oral cavity, and pharynx cancers (LOCPs) in Chinese adults. Methods: Based on the baseline survey and long-term follow-up of the China Kadoorie Biobank (CKB) study, Cox proportional hazard regression models were used to estimate hazard ratios (HR) and 95% confidence intervals (95%CI) for associations between spicy food consumption and LOCPs incidence. Results: Of the 510 145 participants included at baseline, 30.1% reported daily spicy food consumption. During a mean follow-up of 10.8 (2.0) years, we documented 767 LOCPs cases. Multivariate adjusted analyses showed that the risk of LOCPs incidence decreased with the frequency of spicy food intake (trend P=0.003), with HR of 0.69 (95%CI:0.54-0.88) for daily spicy food consumers, compared with never or occasional consumers. Participants who preferred moderate pungency degrees had the lowest risk of LOCPs, with a 33%[0.67(95%CI:0.52-0.87)] reduced risk compared to those who consumed spicy food less than once per week. The later the starting age, the lower the risk (trend P=0.004). Those who started eating spicy food after 18 years old had the lowest risk of LOCPs incidence, with adjusted HR (95%CI) of 0.70(0.54-0.92). Conclusions: Spicy food intake might be associated with a decreased risk of LOCPs incidence. Such association was independent of healthy lifestyles. Advocating moderate-pungency spicy food consumption and healthy lifestyles might help prevent LOCPs.

Age-related changes in liver metabolism and antioxidant capacity of laying hens.

This study aimed to investigate the age-related changes of hepatic metabolism and antioxidant capacity of laying hens at 3 different ages. A total of 192 Hy-line Brown laying hens were assigned into 3 groups: 1) 195-day-old (D195 group); 2) 340-day-old (D340 group); 3) 525-day-old (D525 group). Each group replicated 8 times with 8 hens at the same age. Higher activity of aspartate aminotransferase and lower contents of total protein and globulin were observed in the serum of 525-day-old hens in comparison with their 195-day-old counterparts (P < 0.05). The 525-day-old hens accumulated higher contents of total cholesterol and triglyceride in the liver than 195-day-old birds. Additionally, compared with hens from D195 or D340 group, 525-day-old birds exhibited a lower circulating estradiol level (P < 0.05). For antioxidant capacity, birds in the D525 group showed a higher malondialdehyde concentration in both serum and liver as compared with D195 or D340 group (P < 0.05). The 525-day-old hens also exhibited lower glutathione peroxidase activities in both serum and liver when compared with 195-day-old birds (P < 0.05). Simultaneously, there was a decline of hepatic superoxide dismutase activity in the D525 group in comparison with D195 group (P < 0.05). Compared with 195-day-old counterparts, 340-day-old birds upregulated the mRNA abundance of nuclear factor erythroid-2 related factor 2 and glutathione peroxidase 1 in the liver (P < 0.05). In contrast, hens from D525 group showed the downregulation of hepatic nuclear factor erythroid-2 related factor 2, NAD(P)H quinone dehydrogenase 1, and superoxide dismutase 1 when compared with D340 group (P < 0.05). These results indicated that increasing age can adversely affect liver metabolism and function of laying hens.

[Mesenchymal chondrosarcoma in central nervous system: a clinicopathological analysis].

Objective: To investigate the clinicopathological features of central nervous system (CNS) mesenchymal chondrosarcoma (MCS). Methods: Nine cases of CNS MCS were collected at the First Affiliated Hospital of Fujian Medical University from September 2010 to September 2020. The clinical,imaging,histopathological and immunohistochemical features were reviewed. NCOA2 gene rearrangement was evaluated by fluorescence in situ hybridization (FISH). Results: There were three male and six female patients, with age range of 1 to 59 years (median 31 years). Six cases were intracranial and three cases were intraspinal, and the tumors showed dural attachment. They were often diagnosed as meningioma basing on preoperative imaging. Microscopically, the tumors showed a characteristic biphasic histologic pattern composed of undifferentiated mesenchymal small cells and well-differentiated hyaline cartilage islands. The small cells area were positive for SOX9 (9/9), CD99 (8/9), and without BRG1 and INI1 deletion. The cartilaginous component expressed SOX9 (9/9) and S-100 protein (8/9). NCOA2 gene break apart signal was identified in five cases (5/5). Eight patients were followed up for 4-124 months. Three patients (3/8) had recurrences within one year and two patients died of the tumor. Conclusions: CNS MCS is an extremely rare malignant neoplasm with a propensity to dural involvement. Preoperative imaging has low diagnostic accuracy. CNS MCS should be differentiated from other CNS small round cell tumors and chondrosarcoma. FISH detection of NCOA2 gene rearrangement will assist the diagnosis of MCS.

[Identification of characteristic methylation sites in gastric cancer using genomics-based machine learning].

Objective: To construct a prediction model of gastric cancer related methylation using machine learning algorithms based on genomic data. Methods: The gene mutation data, gene expression data and methylation chip data of gastric cancer were downloaded from The Caner Genome Atlas database, feature selection was conducted, and support vector machine (radial basis function), random forest and error back propagation (BP) neural network models were constructed; the model was verified in the new data set. Results: Among the three machine learning models, BP neural network had the highest test efficiency (F1 score=0.89,Kappa=0.66, area under curve=0.93). Conclusion: Machine learning algorithms, particularly BP neural network, can be used to take advantages of the genomic data for discovering molecular markers, and to help identify characteristic methylation sites of gastric cancer.

[Clinicopathological features of adenocarcinoma of the rete testis].

Objective: To investigate the clinicopathological features, immunophenotype, and differential diagnosis of adenocarcinoma of the rete testis. Methods: Four adenocarcinoma cases of the rete testis diagnosed at West China Hospital, Chengdu, China (3 cases, including 2 consultation cases) and the First Affiliated Hospital of Fujian Medical University, Fuzhou, China (1 case) between January 2009 and December 2017 were included. Their clinical, morphologic and immunohistochemical features were analyzed using histological analysis and immunohistochemical staining. Related literature was reviewed to reveal the characteristics of this tumor. Results: The 4 patients' age range was 26-64 years. The maximum diameters of the tumors were 3.0 and 4.5 cm in 2 cases, respectively. On gross examination, adenocarcinomas of the rete testis appeared as a solid, white to gray or tan to yellow mass that raised at the hilum of the testis. Microscopically, all tumors showed multiple histologic patterns, including corded/trabecular (4/4), glandular, nested, sarcomatoid (3/4), solid (2/4), papillary, cribriform, and slit-like (1/4). Three types of adenocarcinoma cells included cuboidal to columnar (4/4), polygonal (4/4) and spindle-shaped (2/4) with pale eosinophilic and clear cytoplasm. The tumor cell nuclei appeared moderately to markedly atypical and pleomorphic, with a various number of mitoses. Transition from benign to malignant rete epithelium was seen in all cases. Eosinophilic hyaloid globules were found in 1 case. On immunohistochemical study, the tumor cells were diffusely, strongly positive for CKpan (4/4), EMA (4/4), Ber-EP4 (3/3) and CAⅨ(2/2), and focally positive for CK7 (4/4), vimentin (4/4), CD10 (4/4), PAX8 (3/3), PAX2 (3/3). The Ki-67 proliferative index was all>50% (4/4). The prognosis was poor. Two of the 3 patients died within 1 year after the surgical resection. Conclusions: Adenocarcinoma of the rete testis is a rare malignant tumor with several histologic patterns. Transition from benign to malignant rete epithelium is an important diagnostic clue. Detailed clinical history, tumor growth site and immunohistochemistry are helpful for its diagnosis and differential diagnosis.

BMSCs attenuate hepatic fibrosis in autoimmune hepatitis through regulation of LMO7-AP1-TGFß signaling pathway.

OBJECTIVE: In a previous study, we reported that transplantation of bone mesenchymal stem cells (BMSCs) significantly attenuated liver damage in a mouse autoimmune hepatitis (AIH) model. Moreover, expression of the LIM domain protein, LMO7, correlated positively with the invasive capacity of hepatoma cells. However, whether LMO7 plays a role in inflammation and fibrosis of AIH remains unknown. This investigation aimed to explore the effect of BMSC transplantation on LMO7 and the role of LMO7 in hepatic fibrosis. MATERIALS AND METHODS: S100-induced murine AIH and LPS-induced hepatocyte injury models were successfully established. Three doses of BMSCs were injected into AIH mice via the tail vein. LPS-treated AML12 cells were co-cultured with BMSCs in vitro. Small interfering (si) LMO7 RNA and T5224 (a specific inhibitor of AP-1) were used to demonstrate the relationship between LMO7-AP1-transforming growth factor (TGF)-ß. RESULTS: Pathological examination and serum alanine and aspartate aminotransferase levels indicated that liver damage was notably ameliorated in the BMSC-treated mice. LMO7 level was upregulated, while AP-1 and TGF-ß levels were downregulated upon intervention with BMSCs. AP-1 expression was upregulated in the siLMO7 group, whereas TGF-ß level was downregulated in the T5224 group when compared to those in the control group. CONCLUSIONS: BMSC transplantation significantly limits liver fibrosis and upregulates the expression of LMO7. LMO7 inhibits the TGF-ß pathway by inhibiting AP-1. This implies that BMSCs are a potential means of treating liver fibrosis. This approach has important implications for the treatment of AIH and other fibrotic diseases.

Dietary squalene supplementation alleviates diquat-induced oxidative stress and liver damage of broiler chickens.

The aim of this study was to explore the protective effects of squalene supplementation on growth performance, oxidative status, and liver function of diquat-challenged broilers. One hundred forty-four 1-day-old male Ross 308 broiler chicks were allocated to 3 groups, and each group consisted of 6 replicates of 8 birds each. The three groups were as follows: 1) nonchallenged broilers fed with a basal diet (control group), 2) diquat-challenged broilers fed a basal diet, and 3) diquat-challenged broilers fed with a basal diet supplemented with 1.0 g/kg of squalene. Broilers were intraperitoneally injected with 20 mg/mL of diquat solution at a dosage of 1 mL/kg of BW or an equivalent amount of saline at 20 d. Compared with the control group, weight gain and BW change rate during 24 h after injection were decreased by diquat challenge (P < 0.05), and the diquat-induced compromised growth performance was improved by squalene supplementation (P < 0.05). Diquat administration reduced plasma superoxide dismutase activity and increased malondialdehyde accumulation and glutathione peroxidase activity in both plasma and the liver (P < 0.05). In contrast, plasma glutathione peroxidase activity in diquat-challenged broilers was reduced by squalene supplementation (P < 0.05). The hepatic glutathione level was reduced by diquat administration (P < 0.05), whereas its level in plasma and the liver of diquat-challenged broilers was increased by squalene supplementation (P < 0.05). The relative liver weight of broilers was increased by diquat challenge (P < 0.05), with its value being intermediate in the squalene-supplemented group (P > 0.05). The plasma aminotransferase activities and total bilirubin concentration were increased by diquat challenge (P < 0.05), which were reduced by squalene supplementation (P < 0.05). The mRNA abundance of hepatic nuclear factor erythroid 2-related factor 2 (P < 0.05) was upregulated by diquat treatment, regardless of squalene supplementation. The mRNA abundance of hepatic glutathione peroxidase 1 and B-cell lymphoma/leukemia 2-associated X protein was upregulated by diquat challenge (P < 0.05), which was reversed by squalene administration (P < 0.05). Squalene increased NAD(P)H quinone dehydrogenase 1 mRNA abundance and decreased caspase 3 mRNA abundance in the liver of diquat-challenged broilers (P < 0.05). The results suggested that squalene can increase weight gain, improve oxidative status, and alleviate liver injury in diquat-challenged broilers.

[Clinical characteristics and outcome of pregnant patients with myasthenia gravis].

Objective: To study the clinical characteristics of pregnant patients with myasthenia gravis (MG) and the influence of MG to pregnancy. Methods: A retrospective study was conducted including 28 MG patients with 38 pregnancies admitted to the 8th Medical Center of PLA General Hospital between January 2013 and October 2018. Data were collected including clinical scores of MG, serum level of acetylcholine receptor (AChR) antibodies, abnormal repetitive nerve stimulation (RNS) and history of thymectomy before pregnancy. The course of pregnancy, delivery and neonatal outcome were also analyzed. According the outcome of MG, patients were divided into three groups, i.e. improvement group, stable group and deterioration group. Results: (1) The age of MG patients ranged from 21 to 36 (27±4) years. The previous course of MG was 0.5-17.2 (7.4±5.8)years. Based on Osserman clinical type, type ⅡA was the most common one [44.1% (15/34)], followed with type Ⅰ [29.4% (10/34)], type ⅡB [23.5% (8/34)] and type Ⅳ (2.9%).(2)There were 38 pregnancies in 28 women whose pregnancy outcomes resulted in one spontaneous abortion, three embryonic arrest and 34 live births. All abortions developed in the first trimester. Among the 34 pregnancies with live births, the symptoms of MG improved in 16 pregnancies (47.1%), whereas those deteriorated in 10 pregnancies (29.4%) during the first or third trimester and remained stable in 8 pregnancies (23.5%). (3) Compared with improvement group and stable group, the deterioration group had shorter duration of MG [(1.1±0.5) years vs. (7.1±5.1) years, (9.0±5.4) years respectively], higher clinical scores (20.9±6.0 vs. 14.8±6.6,13.3±5.0) and more frequent abnormal RNS(9/10 vs. 8/16, 4/8) and type ⅡB(6/10 vs. 1/16, 1/8) before pregnancy. Positive rate of serum AChR antibody and percentage of thymectomy before pregnancy were comparable between three groups. (4) Spinal anesthesia was performed in 23 pregnancies and 11 cases were vaginal delivery. No transient neonatal MG were found in live-born infants. Conclusions: Pregnancy in patients with under-controlled myasthenia gravis should not be discouraged. The outcome of MG is affected by the duration of MG, MG score and RNS before pregnancy. The first and third trimesters of pregnancy are considered high-risk periods for MG exacerbations. Neonatal transient myasthenia is uncommon, but the newborn should be carefully monitored by obstetricians and neurologists.

[Research progress of prognostic analysis and clinicopathological characteristics of hepatoid adenocarcinoma of the stomach].

Hepatoid adenocarcinoma of the stomach is a rare type of gastric cancer. Compared to the common gastric adenocarcinoma, the tumor markers, imaging manifestation, histopathological features, immunophenotype, treatment and prognosis of HAS are distinct. It has characteristics of gastric adenocarcinoma and hepatocellular carcinoma at the same time, which leads to the misdiagnosis and missed diagnosis. The purpose of this paper is to analysis the characteristics of HAS from the above aspects, improve the diagnosis rate, reduce mortality rate and prolong the survival period of HAS patients.

[The role of SMARCE1 in the diagnosis of clear cell meningioma].

Objective: To analyze the expression of SMARCE1 in clear cell meningioma (CCM), and evaluate the role of SMARCE1 in the differential diagnosis in morphologically similar diseases. Methods: Thirteen samples/11 cases of CCMs were collected from the First Affiliated Hospital of Fujian Medical University, Shandong Provincial Hospital, Xuanwu Hospital of Capital Medical University and Thaihe Hospital of Hubei Province from January 2000 to December 2018, as well as 17 cases of meningiomas with clear-cell-like morphology, 782 cases of other types of meningiomas and other intracranial tumors with clear-like morphology. A tissue microarray was made using these cases, on which immunohistochemical/histochemical staining of SMARCE1, SSTR2, EMA, Ki-67, p53, PAS and D-PAS were performed. Result: The tumor cells of CCM had sheet-like architecture, without typical whorl formation.The CCM had round to polygonal cells, with clear, glycogen-rich cytoplasm and prominent blocky perivascular and interstitial collagen. The immunohistochemistry staining showed that none of the CCMs expressed SMARCE1(0/13).However, all of the other types of lesions, including meningioma(782/782), meningiomas with clear-like morphology(17/17), intracranial metastatic clear cell renal cell carcinoma(10/10), haemangioblastoma(10/10), central neurocytoma(10/10), oligodendroglioma(10/10), ependymoma(13/13), lioblastoma(42/42), and solitary fibrous tumor/hemangiopericytoma(35/35) showed positive nuclear staining of SMARCE1. Ki-67 index were 1%-5%, and p53 positive-rate were 0-40% in CCMs. PAS stain showed cytoplasmic granular positive and D-PAS were negative in all CCMs and meningiomas with clear-like morphology. Conclusion: SMARCE1 is a useful marker for the diagnosis of CCM and its mimickers.

[Primary skull base chondrosarcoma: a clinicopathological analysis].

Objective: To investigate the clinicopathological features, immunophenotype, molecular characteristics and differential diagnosis of primary skull base chondrosarcoma. Methods: Nine cases of primary skull base chondrosarcoma were collected at the First Affiliated Hospital of Fujian Medical University, from January 2006 to June 2019, reviewed for the clinical and radiologic data and morphologic features, immunophenotype and molecular characteristics. Results: Among all the 9 cases, six were male, three were frmale, with average age 47 years, and median age 47 years; five cases were WHO gradeⅠ, and four were WHO grade Ⅱ. Microscopically, the tumor showed lobulated growth pattern with low-medium cellularity within a chondroid or mucoid background. The tumor cells showed mild-moderate atypia, with binucleated forms, and mitosis was rare or occasional. Immunohistochemistry (IHC) showed tumor cells were positive for S-100 protein, vimentin, SOX-9 and D2-40, and negative for Brachyury, CK, EMA and CK8/18; the Ki-67 index was low (1% to 5%). Molecular analysis showed IDH1 R132C mutation in four cases. Conclusions: Skull base chondrosarcoma is a rare cartilaginous malignant tumor with a good prognosis. Its characteristic morphologies, combined with IHC and molecular detection are helpful for the differential diagnosis.