Objective: To investigate the clinical features, immunohistochemical and differential diagnosis of desmoplastic fibroblastoma. Methods: The clinical data and pathology features of 7 cases of desmoplastic fibroblastoma were collected and immunohistochemical study were carried out in all cases with a review of the literatures. Results: There were 2 males and 5 females, with age ranging from 31 to 71 years (average and mean age were 59 and 61 years, respectively). The tumors were located in extremities and abdomen (left toe and right toe, right foot back, left leg and right thigh, right forearm and left hepatic lobe). Clinically, the tumors presented as slow growing painless masses of long standing duration. Grossly, the tumors were well-circumscribed with firm, white to gray cut-off surface. Tumor size ranged from 1.2 to 4.0 cm in maximum diameter (average 3.0 cm). Microscopically, 2 cases were located in dermis, 4 cases were located in subcutaneous and 1 case was located in liver parenchyma. It was composed of spindle-shaped or stellate cells with a fibroblastic or myofibroblastic appearance, and sparsely scattered in densely fibrous or fibromyxoid background. There was small vascular component in tumor background. At high magnification, the tumor cells were medium size with abundant cytoplasm, and the nucleus were small and always with small nucleoli. In some cases, the tumor cells were slightly larger with enlarged nuclei, but without cellular atypical and mitosis. Immunohistochemical study showed that the tumor cells were strongly positive for vimentin, desmin, S-100 protein and CD34, but CKpan was negative. α-SMA showed focal positive in one case. Ki-67 index ranged from 1% to 2%. Four cases were followed-up (ranged from 11 to 21 months, average 16.5 months) and the patients had no recurrence after surgery. Conclusions Desmoplastic firoblastoma is a rare soft benign tumor. The differential diagnosis includes other benign or low-grade fibroblastic/myofibroblastic lesions.

Objective To investigate the effect of optimizing protocol activity on the cardiac function in patients with hypertension.Methods One hundred and one patients with mild and moderate hypertension in Navy General Hospital were enrolled in the study.The patients took regular exercise(6 min walking activity and treadmill test),Echocardiogram was performed 24 h after 6 min walking activity and treadmill test.The cardiac function related parameters were obtained through M-mode,2DE,pulse Doppler and DTI detection to compare the effect of 6 min walking activity and treadmill test on cardiac function.Results The results showed that in the male hypertensive patients,EF and E/E' after treadmill test were superior to those after 6 min walking activity (EF:(59.33±4.46)% vs.(56.05±4.57)%;E/E':(4.12±1.66)vs.(4.95±1.79)),the differences were statistically significant(P=0.02,0.01),while among the female hypertensive patients,there were no significant differences in cardiac function(P>0.05).Conclusion Appropriate activity can improve the cardiac function in male hypertensive patients.EF and E/E' after the treadmill test performed better than those after the 6 min walking activity,while its influence on female hypertensive patients was not significant.

OBJECTIVETo study the clinicopathological characteristics of hepatic angiomyolipoma (HAML) and to evaluate the correlation between clinicopathological parameters and tumor subtypes.

METHODSRetrospective analysis of clinicopathological features was conducted in 182 cases of HAML.

RESULTSHAML patients were predominantly female (M:F=1:4) and most commonly presented with non-specific symptoms. The median age at diagnosis was 46 years, ranged from 17 to 77 years. Tumor diameter was ranged from 0.3 to 32.0 cm with an average of 5.0 cm. Majority of the tumor was epithelioid type (112/165, 67.9%). Extramedullary hematopoiesis, multinucleated giant cells, intranuclear inclusions, nucleolus, cellular atypia, invasive growth pattern, multiple masses, hyperpigmentation and purpura-like changes mostly occurred in the epithelioid type (P<0.05). Extramedullary hematopoiesis was commonly seen in HAML, the significance of which was still uncertain.

CONCLUSIONSMost of HAML are epithelioid type, characterized by a proliferation of predominantly epithelioid cells, in which extramedullary hematopoiesis is commonly seen. Some morphologic features that may predict malignant such as necrosis, mitotic figures, and tumor emboli are only found in the epithelioid HAML. Mitotic activity, tumor necrosis, tumor thrombus, giant cells, periportal invasion, multiple lesions and tumors size over 10 cm are closely related with tumor recurrence and metastasis.

Adolescent ; Adult ; Aged ; Angiomyolipoma ; diagnosis ; pathology ; Epithelioid Cells ; cytology ; Female ; Gastrointestinal Neoplasms ; diagnosis ; pathology ; Giant Cells ; pathology ; Humans ; Middle Aged ; Necrosis ; Neoplasm Recurrence, Local ; Prognosis ; Retrospective Studies ; Young Adult

OBJECTIVE To explore the genetic mechanism for a family affected with cardiac conduction block. METHODS Affected family members were screened for potential mutations of known candidate genes. As no pathogenic mutation was found, two patients and one healthy member from the family were further analyzed by exomic sequencing followed by Sanger sequencing. The pathogenicity of suspected mutation was analyzed using bioinformatics software. RESULTS Sequencing of the full exome has identified a c.G1725T mutation in the CLCA2 gene. Sanger sequencing has detected the same mutation in all five patients, but not in the normal member from the family. Bioinformatics analysis indicated that the mutation has resulted in substitution of the 575th amino acid cysteine (C) by tryptophan (W). The site is highly conserved and becomes pathogenic with the mutation. CONCLUSION The heterozygous c.G1725T mutation in exon 11 of the CLCA2 gene probably underlies the disease and fit the autosomal dominant pattern of inheritance.
Amino Acid Sequence ; Chloride Channels ; genetics ; Computational Biology ; Female ; Heart Block ; genetics ; Humans ; Male ; Middle Aged ; Molecular Sequence Data ; Mutation

Adult ; Hamartoma ; pathology ; Humans ; Liver ; pathology

Objective:To study the clinicopathologic features of IgG4-related pulmonary inflammatory pseudotumor (IRPIP), and its correlations with CD4+T lymphocytes and interleukin-10 (IL-10) expression,and to investigate the immunopathological factors in the disease of IgG4-related pulmonary inflammatory pseudotumor .Methods: Expressions of IL-10, CD3, CD20, CD4, CD8 were examined by immunohistochemistry in 17 cases of IgG4-related pulmonary inflammatory pseudotumor tissues and 15 cases of IgG4-nonrelated pulmonary inflammatory pseudotumor tissues ( INPIP) .To analyze the differences of these indexes expression between IRPIP and INPIP.Results: In group of IRPIP, the CD4+T lymphocytes number and the ratio of CD 4+T lymphocytes number/CD8+T lymphocytes number were all apparently higher than those in the group of INPIP (P=0.006,0.023,respectively).The cells numbers of CD3,CD8,IL-10 positive expression in IRPIP were higer than those in INPIP ,and the cells number of CD20 positive expression in IRPIP was lower than that in INPIP,but these differences all had no statistical significance (P>0.05).In IRPIP,the number of IgG4+plasmacytes was positively related to the number of IL-10+cell (r=0.517),the correlation had statistical significance (P=0.033). Conclusion:The number of CD4+T lymphocytes increased in addition to the increased number of IgG 4+plasmacytes in the lesion back-ground of IRPIP,and the number of IgG4+plasmacytes was positively related to the number of IL-10+cells.It is suggested that the increased number of Th 2 lymphocyte and the increased secretion of cytokine IL-10were are immunopathological factors participated in the disease of IRPIP .

AIM:To select an efficient way of promoting induced pluripotent stem cells ( iPSC) to differentiate into neural stem cells (NSC) by comparing 2 methods.METHODS:The culture system in method A contained SB431542 (5 mmol/L) and drosomophorin (5 mmol/L) with 100%initial cell density, while that in method B contained SB431542 (5 mmol/L) and drosomophorin (1 mmol/L) with 30%~50% initial cell density.For comparison and identification of the 2 methods, the growth state was observed under microscope , and the expression of Pax6, nestin, Sox1 and Sox2 was quantitatively detected by real-time PCR and flow cytometry .The related protein expression and the ability of spontaneous differentiation were determined by immunofluorescence analysis .RESULTS: The cells derived from method A with 5 mmol/L of SB431542 and drosomophorin and 100% initial cell density achieved the higher expression of Pax 6, nestin, Sox1 and Sox2.The growth state was better and the cells differentiated into neurons and astrocytes normally .CONCLU-SION:The method A was superior to method B , and we recommend the method A with 5 mmol/L of SB431542 and droso-mophorin and 100%initial cell density as the method for differentiating NSC .

OBJECTIVETo study the clinical features, pathologic findings and prognosis of patients with dysplastic nodules of liver (DN) and early hepatocellular carcinomas (eHCC).

METHODSOne hundred and forty-five archival cases previously diagnosed as DN or eHCC or well-differentiated HCC during the period from 2000 to 2009 were retrieved and reevaluated with the new diagnostic criteria by two experienced pathologists, according to International Consensus Group for Hepatocellular Neoplasia (ICGHN) 2008. Immunohistochemical study (EnVision method) for CD34, HSP70, glutamine synthetase, glypican 3 and Ki-67 was carried out. The original diagnosis and diagnosis after review were compared and correlated with the survival data of the patients, with statistical analysis.

RESULTSWith the new criteria, 16 cases were diagnosed as low-grade DN, 50 cases as high-grade DN, 72 cases as DN with microinvasion, 7 cases as advanced HCC. Slide review showed no diagnostic discrepancy in 112 cases (77.2%). Amongst the 33 (22.8%) underdiagnosed cases, there were 7 cases of advanced HCC initially diagnosed as DN or DN with microinvasion and 26 cases of eHCC initially diagnosed as high-grade DN. Kaplan-Meier analysis showed that the diagnosis of high-grade DN or early HCC carried no statistically significant difference in overall survival (P = 0.778, 0.677) or disease-free survival (P = 0.949, 0.700) in all patients and in patients with no history of HCC. The co-existence of advanced HCC in patients with DN or eHCC significantly correlated with overall survival (P = 0.004) but not with disease-free survival (P = 0.079).

CONCLUSIONSThe new diagnostic criteria by ICGHN 2008 are useful in delineating high-grade DN and eHCC. The overall survival and disease-free survival of patients with eHCC or high-grade DN undergoing hepatectomy show no statistically significant difference. Patients with DN or eHCC have better prognosis than patients with advanced HCC, though there is still a high risk of tumor recurrence.

Antigens, CD34 ; metabolism ; Carcinoma, Hepatocellular ; metabolism ; pathology ; surgery ; Cell Transformation, Neoplastic ; Disease-Free Survival ; Female ; Follow-Up Studies ; HSP70 Heat-Shock Proteins ; metabolism ; Hepatectomy ; Humans ; Kaplan-Meier Estimate ; Ki-67 Antigen ; metabolism ; Liver Cirrhosis ; metabolism ; pathology ; surgery ; Liver Neoplasms ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Survival Rate

BACKGROUNDThe MAPK phosphatases (MKPs) are a family of dual-specificity phosphatases (DUSPs) that can dephosphorylate both phosphothreonine and phosphotyrosine residues, thus inactivating MAPK signaling. DUSP6 is a cytoplasmic MKP that can inactivate ERK. DUSP6 has been implicated in the development of some tumors. The aim of this research was to investigate the expression of DUSP6 in hepatocellular carcinoma (HCC) and the correlation of DUSP6 with mitogen-activated protein kinases (MAPKs), clinicopathological characteristics, and prognosis.

METHODSTissues from 305 patients who had undergone hepatectomy for HCC was used in this study. The expression of DUSP6, p-ERK, p-JNK, and p-p38α was determined using tissue microarrays for immunohistochemical analysis. The prognostic value of DUSP6 and other clinicopathological factors were evaluated.

RESULTSThe expression of DUSP6 was significantly higher in the tumor tissue when compared to the peritumor or normal liver tissue (P < 0.001). Tumor DUSP6 expression was significantly associated with disease-free survival (DFS) (P = 0.013). Tumor DUSP6 expression was an independent prognostic factor for DFS (Hazard ratio = 1.635, P = 0.006).

CONCLUSIONSDUSP6 is over expressed in tumor tissue compared to peritumor or normal liver tissue. Higher expression of DUSP6 in tumor tissue, than in peritumor tissue, is associated with the recurrence after curative resection of HCC, and the relative tumor DUSP6 expression has good power to predict the recurrence of HCC.

Adult ; Aged ; Aged, 80 and over ; Carcinoma, Hepatocellular ; metabolism ; Dual Specificity Phosphatase 6 ; metabolism ; Female ; Humans ; Immunohistochemistry ; Liver Neoplasms ; metabolism ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; metabolism ; Tissue Array Analysis ; Young Adult

BACKGROUND:Many studies have showed that neural stem cells therapy is a new strategy for hypoxic-ischemic encephalopathy.
OBJECTIVE:To review and analyze the status of research, transplantation strategies and mechanism of neural stem cells therapy for treatment of hypoxic-ischemic encephalopathy.
METHODS:A computer-based retrieval was performed in PubMed and CNKI database to search papers published from August 2000 to August 2013 using the key words of“hypoxic-ischemic encephalopathy, neural stem cells”in English and Chinese. The papers with objective-independent and repetitive contents were excluded, and final y 39 papers were included for final analysis.
RESULTS AND CONCLUSION:Neural stem celltransplantation can promote recovery of neurological function, which brings new hope to hypoxic-ischemic encephalopathy patients. But the study is at a primary stage and limited in laboratory. There are many critical factors that hinder the clinical transplantation, such as delivery path, transplantation time, single or combined transplantation, mechanisms of action. Application of neural stem cells requires further investigation.