Atypical teratoid/rhabdoid tumors of central nervous system in childhood: a clinical and histopathologic study of 6 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunohistochemical findings, diagnosis and differential diagnosis of atypical teratoid/rhabdoid tumors (AT/RT) of central nervous system in childhood.</p><p><b>METHODS</b>The clinicopathologic data, morphologic features and immunophenotypes were reviewed in 6 cases of AT/RT. EnVision method was applied. Antibodies include cytokeratin (CK), epithelial membrane antigen (EMA), vimentin, smooth muscle actin (SMA), muscle specific actin (MSA), glial fibrinary acid protein (GFAP), desmin, placental alkaline phosphatase (PLAP) and INI1.</p><p><b>RESULTS</b>Five of the six cases of AT/RT occurred in infancy and early childhood. Histologically, the predominant component was rhabdoid cells. Cytoplasmic inclusions were present in all cases. Primitive neuroectodermal tumor (PNET) component was also identified in 5 of the 6 cases studied. Immunohistochemically, the tumor cells were positive for cytokeratin, epithelial membrane antigen and vimentin. The staining for INI1, desmin and PLAP was negative. Smooth muscle actin was expressed in 2 cases and glial fibrillary acidic protein in 5 cases. The proliferative index as demonstrated by Ki-67 staining was high.</p><p><b>CONCLUSIONS</b>AT/RT is not a particularly uncommon malignancy in childhood. The histologic hallmark is the presence of rhabdoid cells with cytoplasmic inclusions. The tumor cells are positive for cytokeratin, epithelial membrane antigen and vimentin, and negative for INI1. Differential diagnosis includes PNET, medulloblastoma and medullomyoblastoma.</p>

Correlation between typing of peripheral neuroblastic tumors and prognosis: a clinicopathologic study of 135 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic characteristics of peripheral neuroblastic tumors and to investigate the prognostic significance of International Neuroblastoma Pathology Classification (INPC).</p><p><b>METHODS</b>One hundred and thirty-five cases of peripheral neuroblastic tumors encountered in Shanghai Children's Medical Center were enrolled into the study. All the cases were classified according to INPC and International Neuroblastoma Staging System (INSS). The follow-up data were analyzed.</p><p><b>RESULTS</b>The consensus diagnoses of the 135 cases were as follows: 80 cases (59.2%) of neuroblastoma, 24 cases (17.8%) of ganglioneuroblastoma, intermixed, 17 cases (12.6%) of ganglioneuroma and 14 cases (10.4%) of ganglioneuroblastoma, nodular. The cases were subdivided into 2 subgroups: favorable histology (number = 90, 66.7%) and unfavorable histology (number = 45, 33.3%). According to INSS, the number of cases in stages I, II, III and IV was 22 (16.3%), 24 (17.8%), 34 (25.2%) and 55 (40.7%), respectively. The survival of peripheral neuroblastic tumors correlated with histologic diagnosis, INPC and INSS (P < 0.05).</p><p><b>CONCLUSION</b>Diagnostic categorization of peripheral neuroblastic tumors according to INPC is of prognostic value.</p>

The cultivation and identification of tumor stem cells from neuroblastoma derived tumor spheres / 癌症

<p><b>BACKGROUND AND OBJECTIVE</b>Since the proposal of the tumor stem cell hypothesis, considerable interest has been devoted to the isolation and purification of tumor stem cells. Tumor stem cell enrichment from primary tumor derived cell spheres has been demonstrated in specific, serum-free media. This goal of this study is to establish a method of cultivating floating tumor spheres from neuroblastoma cells and to confirm that neuroblastoma spheres are rich in tumor stem cells.</p><p><b>METHODS</b>Bone marrow aspirates were obtained from pediatric patients diagnosed with stage IV neuroblastoma. Primary tumor cells were isolated and cultivated in serum-free, stem cell-selective medium. Single sphere-forming cells were cultivated under serum-free conditions; their cloning efficiency and monoclonal tumor sphere formation rates were calculated. The expression of stem cell marker genes Oct-4 and Bmi-1 was detected by RT-PCR in sphere-forming cells and parental neurolastoma cells. Sphere-forming cells were injected into the armpit of nude mice with subsequent assessment for tumor growth. Sphere-forming cells were cultivated in differentiation medium containing 5 μmol/L 13-cis retinoic acid; changes in cell morphology were observed.</p><p><b>RESULTS</b>Neuroblastoma cells formed non-adherent neurospheres under serum-free, stem cell-selective conditions after a period of 4 to 6 days. A single cell dissociated from a neurosphere could reform a monoclonal sphere; cloning efficiency and monoclonal sphere formation rates were 55.3% and 26.3%, respectively. RT-PCR results revealed heightened tumor sphere expression of Oct-4 and Bmi-1 as compared with parental tumor cells. Fourteen days after injection of 10(4) sphere-forming cells into nude mice, a neuroblastoma xenograft formed. Treatment of sphere-forming cells with 13-cis retinoic acid induced a gradual differentiation to neuronal cell morphology.</p><p><b>CONCLUSIONS</b>Neuroblastoma derived tumor spheres enrich tumor stem cells and the cultivation of primary neuroblastoma cells in serum-free, stem cell-selective medium is an effective method to dissociate and purify tumor stem cells in vitro.</p>

Relationship between the types of Helicobacter pylori strains and chronic upper gastrointestinal diseases in children / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the relationship of the types of Helicobacter pylori (H. pylori) strains with the classification and the severity of chronic gastro-duodenal diseases in children.</p><p><b>METHODS</b>One hundred and fifteen children with chronic upper gastrointestinal symptoms who were diagnosed as H. pylori infection by gastroscopy were enrolled in this study. H. pylori strains were serotyped by immunoblot technique. The gastric biopsy specimens of all patients were studied histologically.</p><p><b>RESULTS</b>Type I H. pylori strains were confirmed in 84 cases (73.0%), intermediate type strains in 21 cases (18.3%), and type II strains in 10 cases (8.7%). Type I H. pylori strains infection caused a moderate gastric mucosal inflammation in 83 cases and a severe inflammation in 1 case. Intermediate type H. pylori strains infection caused a moderate gastric mucosal inflammation in 21 cases. Type II H. pylori strains infection caused a mild gastric mucosal inflammation in 2 cases and a moderate inflammation in 8 cases. Different types of H. pylori strains resulted in different severity of gastric mucosal inflammation (x2=15.444, P < 0.01). The gastric mucosal inflammation due to type I H. pylori strains was the most severe, while the inflammation due to type II H. pylori strains was relatively mild. The incidence of nodulus lymphaticus of gastric mucosa due to type I, type II and intermediate type H. pylori strains infection was 76.2%, 47.6% and 40.0%, respectively (x2=10.171, P < 0.01). The classification of chronic gastro-duodenal diseases was not associated with the types of H. pylori strains.</p><p><b>CONCLUSIONS</b>Type I strains were the leading cause of H. pylori infection in children. All of types of H. pylori strains can cause pathohistologic changes of gastric mucosa. Type I H. pylori strains infection can result in the most severe gastric mucosal inflammation and the highest incidence of nodulus lymphaticus. The immunoblot serotyping of H.pylori strains may be useless for the classification of chronic upper gastrointestinal diseases but it is helpful for the evaluation of the severity of the diseases in children.</p>

Unusual expression and molecular mechanisms of E-cadherin, beta-catenin in correlation with clinicopathologic parameters in neuroblastoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the expression of E-cadherin and beta-catenin in neuroblastomas of various degrees of differentiation, and to investigate their molecular mechanisms in correlation with clinicopathologic parameters.</p><p><b>METHODS</b>Immunohistochemistry EnVision method was used to detect E-cadherin and beta-catenin expression in 90 paraffin-embedded tissue samples of neuroblastomas. The methylation status of CpG islands of E-cadherin promoter was investigated by MSP in 7 fresh tissue and 24 paraffin-embedded tissue samples. The mutation status of exon 3 of beta-catenin gene was studied by PCR in 7 fresh tissue samples. Statistical analysis of the data was performed by SPSS software.</p><p><b>RESULTS</b>E-cadherin and beta-catenin were abnormally expressed in neuroblastomas in general. The expression of beta-catenin in well-differentiated neuroblastoms was markedly higher (47/70, 67.1%) than that of the poorly differentiated tumors (8/20, 40.0%). There was a markedly decreased expression of both genes in tumors with lymph node metastasis than those without. Demethylation was seen in some regions of the promoter of E-cadherin in 31 cases of nuroblatomas. PCR of the exon 3 of beta-catenin followed by DNA sequencing demonstrated rearrangements and mutations in 7 cases, including 2 cases harboring identical point mutation at gene position 27184, leading to a T-->A alteration.</p><p><b>CONCLUSIONS</b>The abnormal over-expression of E-cadherin in neuroblastomas is independent of the methylation status of their promoter sequences. The abnormal expression of beta-catenin may be related to mutational changes at exon 3 of the gene.</p>

Endoscopic and histopathological characteristics of chronic gastritis,duodenitis and peptic ulcer in children / 上海第二医科大学学报

Objective To investigate the relationship between the endoscopic characteristics of chronic gastritis,duodenitis,peptic ulcer and their histopathologic findings in children,and explore the relationship between Helicobacter pylori(Hp) infection and the severity of histopathologic changes of gastroduodenal mucosa in children. Methods Three hundreds and sixty-six children with chronic upper gastrointestinal symptoms who were examined by gastroscopy were enrolled.The gastric and duodenal mucosal biopsy specimens of all the patients were studied histopathologically. ResultsTypes of chronic gastroduodenal diseases in all these patients were: chronic gastritis(n=206,56.3%),chronic gastritis combined with duodenitis(n=112,30.6%),chronic gastritis combined with peptic ulcer(n=48,13.1%).There was chronic inflammation in gastric mucosa and duodenal bulb mucosa in all the cases examined by histopathologic examination.Hp infection was found in 106 cases(28.96%).The gastric mucosal inflammation was more severe in those with Hp infection than those without(P0.05).The were significant differences in the incidences of inflammation activity,atrophia and nodulus lymphaticus of gastric mucosa between those with Hp infection and those without(P

Expression of ?-catenin in keratosic dermatosis and non-melanocytic skin tumors / 上海第二医科大学学报

Objective To study the expression and significance of ?-catenin in keratosic dermatosis and non-melanocytic skin tumors. Methods ?-catenin expression was determined in 10 specimens of normal skin,10 cases of seborrheic keratosis(SK),26 cases of actinic keratosis(AK),17 cases of Bowen's disease(BD) and 35 cases of primary cutaneous squamous cell carcinoma(SCC) by immunohistochemical EnVision two-step staining method. Results The expression of ?-catenin was significantly higher in SK,AK,BD and SCC than in normal skin(P0.05),but that was significantly lower in SK and AK than in BD(P0.05). Conclusion The cytoplasmic and/or nuclear expression of ?-catenin may play a role in the progression from normal skin to keratosic dermatosis and non-melanocytic skin tumors.

Analysis of Clinical Characteristic Pathology and Prognosis in Infant with Intra-Abdominal Solid Tumor / 实用儿科临床杂志

ObjectiveTo analyze the relationship between clinical characteristic pathology and prognosis in infant with intra-abdominal solid tumor.MethodsFifty-two infants(less than 1 year old) with abdominal solid tumor from Apr.1998 to Feb.2007 in Shanghai Children's Medical Center and from Jan.2001 to Feb.2007 in Shanghai Xinhua Hospital were reviewed.The history of these children were reviewed.Features and clinical pathology of these children with their prognosis were analyzed and followed up by telephones and children return visit records from 5 months to 8 years.ResultsLess than 1 month,7 cases;1 month to 1 year old,45 cases.Teratoma 23 cases(44.23%),neuroblastoma 9 cases(17.31%),nephroblastoma 6 cases(11.54%),hepatoblastoma 5 cases(9.62%),epithelioid hemangioendothelioma of the liver 3 cases(5.77%),congenital mesoblastic nephroma 3 cases(5.77%),fusiform cell epithelioid hemangioendothelioma of pancreas 1 case(1.92%),hamartoma of the liver 1 case(1.92%),retroperitoneal small cell malignant tumor 1 case(1.92%).Benign:malignant=1:1.Among the benign tumor,male:female=1:1.Among the malignant tumor,male:female=2.33:1.0.All children were treated with tumor resection,and combined with chemotherapy for those whose tumors were malignant.ConclusionsAmong infant abdominal solid tumors,teratoma and neuroblastoma are much more than other tumors.The cases of benign tumors are almost as much as the malignant tumors.The benign tumors did not have sex differences,and had good prognosis after surgical resection.However,in malignant tumors,the incidence rate of male is obviously higher than female.Completely resection of those malignant tumors with chemotherapy would get little incidence of recrudescence and low case fatality rate.Early diagnosis and early treatment play an important role in prognosis.

Metanephric stromal tumor: a pathologic study of two cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features of metanephric stromal tumor (MST), with emphasis on diagnostic criteria.</p><p><b>METHODS</b>The clinicopathologic findings in 2 cases of MST were analyzed and the literature of this entity was reviewed.</p><p><b>RESULTS</b>Cases of MST were unilateral and mostly centered in renal medulla. The tumor was separated from adjacent renal tissue by sharp and scalloped borders. Entrapped tubules and glomeruli were commonly seen within the lesion. The tumor cells were spindle to stellate in shape and arranged in a nodular pattern. On low power examination, alternating areas of high and low tumor cellularity were noted. Characteristically, there were onion skin-like concentric cuffs of tumor cells around entrapped tubules. The small intratumoral vasculatures showed irregular thickening ("angiodysplasia"). Immunohistochemical study demonstrated that the tumor cells diffusely expressed CD34.</p><p><b>CONCLUSIONS</b>Which the tumor cells around the entrapped renal tubules and blood vessels imparts a nodular appearance, as well as the tumor cells labbed for CD34 are the highly characteristic pathologic findings of MST.</p>

Expression of Vascular Endothelial Growth Factor- C Related Metastasis in Solid Tumors of Children / 实用儿科临床杂志

Objective To evaluated the expression of vascular endothelial growth factor -C(VEGF-C) in children with neurob-lastomaor willms' tumors. Methods The specimens from 33 cases with neuroblastoma and 30 cases with willms' tumors were detected by immunohistochemical staining method. Results In thirty - three cases of neuroblastoma, stage III VEGF - C positive rate were 37.5% (3/8),stage IV were 32% (8/25);favourable histological(FH) were 45%(9/20),unfavourable favourable histological(UFH) were 30.8%(4/13).Cases with distant meta stasis were 32% (8/25),and cases with lymph node involvement were 66.7%(4/6). In 30 cases with willms' tumors,stage I + II were 26.7% (4/15),while stage III + IV were 40% (6/15), FH were 27.3% (6/22), while UFH were 25% (2/8). Cases with distant metastasis were 33.3% (4/12),and cases with lymph node involvement were 60% (3/5). VEGF-C positive expression rate was significantly higher in tumors from children with lymph node metastasis than those without. Conclusion The study shows that expression of VEGF -C is related to lymph node metastasis.

Application of Immunohistochemistry Study on Diagnosis of Hirschsprung's Disease / 实用儿科临床杂志

Objective To observe the expression of various antibodies on resection specimens with Hirschsprung's disease(HD), and to find out a special antibody to help the diagnosis of HD. Methods Neuron specific enolase(NSE),S100, Cathepsin D (CAD), Peripherin, protein gene prosuct 9. 5(PGP9. 5) were used as the first antibody to do the EnVision immunohistochemical staining on each end of the resection specimens with HD. Results PGP9.5, NSE, Peripherin were positive both in neuroganglion cells and neuro-plexus in the submucosa and intermuscule; S100 was positive in neutoplexus but negative in neuroganglion cells in the submucosa and intermuscule; CAD was positive in neuroganglion but negative in neuroplexus cells in the submucosa and intermuscule. Conclusion Compared with the other antibodies,CAD is a special antibody which may help the diagnosis of HD; CAD staining results is easy to judge, and it is more faster and economic.