Objective To explore the significance of MYCN gene amplification in children with neuroblastic tumors(NT).Methods The clinicopathological data of 154 cases with NT were reviewed,including general data, classification of pathology,clinical stage and prognosis.MYCN gene amplification was detected by fluorescence in situ hybridization(FISH) and its relationship between pathological characteristics and prognostic significance was analyzed.Results There was 154 cases of NT aged 1 day to 11 years,with a mean age of 26.1 months,and the median age of 20.5 months.Male and female ratio was 1.48 : 1.00.According to International Neuroblastoma Staging System (INSS) ,20 cases were of stage Ⅰ (13.0％) ,23 cases of stage Ⅱ (14.9％) ,43 cases of stage Ⅲ (27.9％) ,64 cases of stage Ⅳ(41.6％) and 4 cases of Ⅳs (2.6％).There were 72 cases(46.8％) with favorable histology,and 82 cases(53.2％) with unfavorable histology.MYCN amplification was found in 20 cases (13.0％) and the signal ratio of MYCN and chromosome 2 (CEP2) was 4.08-43.29.One hundred and thirty-four cases of MYCN non-amplification included MYCN gain in 91 cases(68.0％) ,MYCN negative in 43 cases(32.0％).MYCN expression showed the significant differences in ages, neuroblastoma type, international neuroblastoma pathology classification (INPC), mitosis karyorrhexis index (MKI), and clinical stages (all P ＜ 0.05).No significant difference was found in gender(P ＞ 0.05).Of 20 MYCN amplification cases,4 cases (20.0％) survived and 16 cases (80.0％) died,and the overall survival rate was 20.0％ (4/20 cases) ,with survival time was (17.10 ± 2.24) months;of 134 MYCN non-amplification cases,96 cases (71.6％) survived and 38 cases (28.4％) died, with survival time of (28.71 ± 1.28)months.Survival analysis showed the cases with MYCN amplification had worse prognosis (x2 =19.596, P ＜ 0.05).Conclusions Patients with MYCN amplification had poorer prognosis and lower incidence of MYCN amplification of pediatric NT was found in China.

Objective To study the clinicopathologic features of pediatric vascular anomalies and application of ISSVA classification. Methods The clinical features, histopathologic findings and immunohistochemical results were analyzed in 117 cases of pediatric vascular anomalies encountered during the period from May 2014 to May 2015.Results A total of 117 cases of vascular anomalies were studied. The age of patients ranged from 18 hours after birth to 11 years ( mean age =34 months and median age =27 months) .There were 73 male patients and 44 female patients, with the male-to-female ratio being 1.7∶1.0.Congenital skin lesions were found in 37 cases ( 31.6%) .The common sites of involvement included head and neck region (46 cases, 39.3%), trunk (28 cases, 23.9%), extremities (14 cases, 12.0%) and internal viscera (31 cases, 26.5%).According to the new ISSVA classification, there were 74 cases of vascular malformations and 43 cases of vascular neoplasms ( ratio=1.7∶1.0 ) .The commonest vascular tumor encountered was infantile hemangioma ( 21 cases, 48.8%) , including 17 cases in proliferative phase and 4 cases in involutive phase.Thirteen cases (23.3%) of congenital hemangioma were found, with 8 cases of rapidly involuting congenital hemangioma and 5 cases of non-involutive congenital hemangioma.Three of the congenital hemangioma occurred in liver. There were 5 cases ( 11.6%) of pyogenic granuloma, 3 cases ( 7.0%) of tufted angioma and 1 case ( 2.3%) of Kaposiform hemangioendothelioma.Amongst the 74 cases of vascular malformations encountered, lymphatic malformation was found in 47 cases ( 63.5%) , venous malformation in 15 cases ( 20.2%) , lymphatic-venous malformation in 11 cases (14.9%) and arteriovenous malformation in 1 case (1.4%).All cases of vascular anomalies were all positive for CD31 on immunostaining.Glut1 and CD15 were positive both in proliferative and involutive phases of the 21 cases of infantile hemangioma, while other vascular tumors and vascular malformations were negative.Forty-seven cases of lymphatic malformation and 11 cases of lymphatic-venous malformation showed D2-40 expression.Focal positivity for D2-40 was demonstrated in 3 cases of tufted angioma and 1 case of Kaposiform hemangioendothelioma.Conclusions Vascular anomalies affecting infants and children include tumors and malformations. Accurate histopathologic diagnosis and ISSVA classification of the various types of vascular anomalies play an important role in clinical management.