Search results for "neuroblast"

Influence of Neuroblastoma Stage on Serum-Based Detection of MYCN Amplification

2009

BACKGROUND: MYCN oncogene amplification has been defined as the most important prognostic factor for neuroblastoma (NB), the most common solid extracranial neoplasm in children. High copy numbers are strongly associated with rapid tumor progression and poor outcome, independently of tumor stage or patient age, and this has become an important factor in treatment stratification. PROCEDURE: By real-time quantitative PCR analysis, we evaluated the clinical relevance of circulating MYCN DNA of 267 patients with locoregional or metastatic NB in children less than 18 months of age. RESULTS: For patients in this age group with INSS stage 4 or 4S NB and stage 3 patients, serum-based determination o…

Perioperative management of hypertensive neuroblastoma: A study from the Italian Group of Pediatric Surgical Oncologists (GICOP)

2017

Background: Hypertension (HT) is rarely reported in patients affected by Neuroblastoma (NB), and management guidelines are lacking. Clinical features and perioperativemedical treatment insuchpatientswere reviewed to1) ascertain whether a shared treatment strategy exists among centers and 2) if possible, propose some recommendations for the perioperative management of HT in NB patients. Methods: A retrospectivemulticenter surveywas conducted on patients affected by NBwho presented HT symptoms. Results: From 2006 to 2014, 1126 children were registered in the Italian Registry of Neuroblastoma (RINB). Of these, 21 with HT (1.8%) were included in our analysis. Pre- and intraoperative HT manageme…

High frequency of subclonal ALK mutations in high risk neuroblastoma patients. A SIOPEN study

2015

Introduction: In neuroblastoma (NB), activating ALK receptor tyrosine kinase point mutations are detected in 8–10% at diagnosis using conventional sequencing. To determine the potential occurrence and the prognostic impact of ALK mutations in a series of high risk NB patients we studied ALK variation frequencies using targeted deep sequencing in samples of patients enrolled in the SIOPEN HR-NBL01 study

[Recent advances on retroperitoneal neuroblastoma]

1998

Neuroblastoma, a malignant tumor of infancy and childhood, has some very interesting peculiars: good prognosis, even with disseminated disease, propensity to occasionally undergo spontaneous regression, its ability to undergo spontaneous or induced differentiation to a benign ganglioneuroma. Neuroblastoma may originate anywhere along the sympathetic nervous system chain. The most common site of primary tumor is, however, within the abdomen either in the adrenal gland or in a paraspinal ganglions. A great deal of progress has been made in advancing the knowledge of human neuroblastoma at the cellular and molecular viewpoint. The genetic predisposition to develop the tumor is clarified, a spe…

The Pvt-1 oncogene is a target of Myc and its expression is deregulated in neuroblastoma cells

2003

Age-dependency of the prognostic impact of tumor genomics in localized resectable MYCN non-amplified neuroblastomas Report from the SIOPEN Biology Gr…

2018

BACKGROUND: Biology based treatment reduction, i.e. surgery alone also in case of not totally resected tumors, was advised in neuroblastoma patients with localized resectable disease without MYCN amplification. However, whether the genomic background of these tumors may influence outcome was unknown and therefore scrutinized in a meta-analysis comprising two prospective therapy studies and a ‘validation’ cohort. PATIENTS AND METHODS: Diagnostic samples were derived from 406 INSS stages 1/2A/2B tumors from three cohorts: LNESGI/II and COG. Genomic data were analyzed in two age groups (cut-off: 18 months) and quality controlled by the SIOPEN Biology Group. RESULTS: In both patient age groups …

Estudio del armazón arquitectónico y del sistema vascular de los tumores neuroblásticos

2015

Los pacientes con tumores neuroblásticos presentan una evolución clínica heterogénea, desde la regresión espontánea hasta una alta propensión para la diseminación metastática generalizada. Aunque la aplicación de una clasificación de riesgo pre-tratamiento bien definida tiene un papel central en la mejora de la supervivencia durante los últimos años, han de llevarse a cabo más avances para mejorar la superviencia de los pacientes en general y específicamente el subgrupo de pacientes de alto riesgo. El estudio morfológico del tejido tumoral está contribuyendo a dicha mejora. La categoría histológica o el porcentaje de estroma tumoral, así como el grado de diferenciación de las células neurob…

Búsqueda de nuevos biomarcadores con utilidad pronóstica en el Neuroblastoma

2016

El neuroblastoma (NB) es un tumor neuroectodérmico de las células embrionarias derivadas de la cresta neural. Constituye el tumor sólido extracraneal más frecuente en la infancia y es altamente heterogéneo en su comportamiento clínico y biológico. Los pacientes considerados de alto riesgo presentan tasas de supervivencia a los 5 años que no superan el 40% en la mayoría de las series estudiadas pese a recibir un tratamiento multimodal de gran intensidad. El resto de pacientes no incluidos en esta categoría son un grupo muy diverso en cuanto a presentación clínica, edad y características moleculares, expuestos a ser sobretratados o infratratados si la asignación de riesgo no está bien definid…

Identification of a novel recurrent 1q42.2-1qter deletion in high risk MYCN single copy 11q deleted neuroblastomas

2012

Neuroblastoma is an aggressive embryonal tumor that accounts for similar to 15% of childhood cancer deaths. Hitherto, despite the availability of comprehensive genomic data on DNA copy number changes in neuroblastoma, relatively little is known about the genes driving neuroblastoma tumorigenesis. In this study, high resolution array comparative genome hybridization (CGH) was performed on 188 primary neuroblastoma tumors and 33 neuroblastoma cell lines to search for previously undetected recurrent DNA copy number gains and losses. A new recurrent distal chromosome 1q deletion (del(1)(q42.2qter)) was detected in seven cases. Further analysis of available array CGH datasets revealed 13 additio…

The Embryonic Central Nervous System Lineages ofDrosophila melanogaster

1997

Abstract In Drosophila, central nervous system (CNS) formation starts with the delamination from the neuroectoderm of about 30 neuroblasts (NBs) per hemisegment. They give rise to approximately 350 neurons and 30 glial cells during embryonic development. Understanding the mechanisms leading to cell fate specification and differentiation in the CNS requires the identification of the NB lineages. The embryonic lineages derived from 17 NBs of the ventral part of the neuroectoderm have previously been described (Bossing et al., 1996). Here we present 13 lineages derived from the dorsal part of the neuroectoderm and we assign 12 of them to identified NBs. Together, the 13 lineages comprise appro…