Search results for "Neuroblastoma"
showing 9 items of 239 documents
Esthesioneuroblastoma presenting with epifora in a young child
1997
Esthesioneuroblastoma (ENB) is an uncommon tumor believed to arise from the olfactory epithelium. 1 This neoplasm has rarely been reported in children, with only 12 cases reported to date among patients younger than 10 years. 2 The usual initial symptom in children, as in older patients, is nasal obstruction or epistaxis 3 ; consequently, the tumor is often first seen by an otorhinolaryngologist. We report a case of ENB in a young child in whom the initial symptom was epiphora; to our knowledge, this initial symptom is previously unreported, and ENB must now be considered in the differential diagnosis of epiphora in childhood. Report of a Case. A white 6-year-old male child was seen on Janu…
CONGENITAL NEUROBLASTOMA: A RARE CASE OF PLURIVISCERAL METASTATIC DISSEMINATION
2021
Neuroblastoma is one of the most common neonatal tumors. It involves children in early infancy and originates from neural crest cells of the adrenal gland medulla or sympathetic glia. We report an unusual case of congenital poorly differentiated neuroblastoma with multiple metastases in the brain and cerebellum. The biophysical profile showed a prominent polyhydramnios at 25.3 weeks of gestation; meantime, the mother was diagnosed with gestational diabetes. The mass was detected during the third ultrasonographic examination at 31.4 weeks of gestation. The fetus lived only one day after birth and the postmortem examinations were performed subsequently. An autopsy was performed both to unders…
Integración de la genómica en la modelización de los tumores neuroblásticos = Integration of genomics in neuroblastic tumors modeling
2015
*Introducción: El neuroblastoma es un tumor maligno embrionario del sistema nervioso simpático con una gran heterogeneidad en la presentación clínica, morfológica y genética y en otras características biológicas. La estratificación de grupos de riesgo pretratamiento se basa en los siguientes factores: edad, estadio, histopatológica del tumor, estado del oncogén MYCN, integridad del brazo cromosómico 11q y ploidía. Por otra parte, los cambios numéricos y estructurales detectados en el perfil genético se han incluido recientemente en la estratificación terapéutica de los pacientes con bajo riesgo a recaer o morir. El amplio espectro clínico de la enfermedad va desde pacientes con neuroblastom…
Transmisión de señales mecánicas en células indiferenciadas neuroblásticas. Estudios biológicos y preclínicos en modelos in vitro 3D
2023
La biotensegridad es un principio físico de autoequilibrio en el que las fuerzas de tensión y compresión sostienen las estructuras biológicas y permiten la mecanotransducción de señales durante las interacciones célula-célula y célula-entorno. De esta manera, las células y los tejidos responden a las condiciones de su entorno y se adaptan a él. En este sentido, el cáncer muestra sus propios sistemas biotensegrales aberrantes que impulsan la carcinogénesis y la evolución de la enfermedad. La biotensegridad tumoral se define por la intercomunicación entre las poblaciones de células tumorales, los elementos de la matriz extracelular y las estructuras y moléculas del microambiente tumoral. En l…
Alteraciones cromosómicas segmentarias como marcador pronóstico en neuroblastoma y sarcomas pediátricos
2020
NEUROBLASTOMA Tumor descrito por primera vez en por el patólogo alemán Virchow en 1864, el neuroblastoma (NB) es el tumor sólido extracraneal más frecuente entre la población pediátrica, y forma parte de los tumores derivados del sistema nervioso simpático (en cadena simpática o ganglios simpáticos). Otros tumores también derivados de la cresta neural son el ganglioneuroblastoma y el ganglioneuroma. Las características del neuroblastoma son las siguientes: - Capacidad de madurar de formas indiferenciadas (neuroblastomas) a formas maduras (ganglioneuroma). - Capacidad de regresar espontáneamente. - Buen pronóstico en pacientes menores de un año. - Comportamiento altamente agresivo en formas …
Evaluación de una puntuación semicuantitativa de MIBG como factor pronóstico en neuroblastoma metastásico y correlación con otros factores pronóstico…
2013
SEMIQUANTITATIVE 123I-METAIODOBENZYLGUANIDINE SCORE AS A PROGNOSIS MARKER IN HIGH RISK NEUROBLASTOMA: CORRELATION WITH OTHER PROGNOSTIC MARKERS AND MINIMAL RESIDUAL DISEASE IN BONE MARROW. Purpose: Metaiodobenzylguanidine (MIBG), specifically taken up in cells of sympathetic origin, provides a highly sensitive and specific marker for the detection of metastases in neuroblastoma. Our aim has been to investigate whether response to induction therapy, evaluated by 123I-MIBG correlates with overall survival (OS) and other prognostic factors in children with metastatic neuroblastoma. Patients and Methods: Medical records and imaging studies from 28 patients with stage 4 neuroblastoma have been r…
Epigenetic siRNA and Chemical Screens Identify SETD8 Inhibition as a Therapeutic Strategy for p53 Activation in High-Risk Neuroblastoma
2017
Given the paucity of druggable mutations in high-risk neuroblastoma (NB), we undertook chromatin-focused small interfering RNA and chemical screens to uncover epigenetic regulators critical for the differentiation block in high-risk NB. High-content Opera imaging identified 53 genes whose loss of expression led to a decrease in NB cell proliferation and 16 also induced differentiation. From these, the secondary chemical screen identified SETD8, the H4K20me1 methyltransferase, as a druggable NB target. Functional studies revealed that SETD8 ablation rescued the pro-apoptotic and cell-cycle arrest functions of p53 by decreasing p53K382me1, leading to activation of the p53 canonical pathway. I…
High-SETD8 inactivates p53 in neuroblastoma
2017
Molecular mechanisms of MYCN-dependent apoptosis and the MDM2-p53 pathway: an Achille’s heel to be exploited for the therapy of MYCN amplified neurob…
2012
The p53 oncosuppressor is very seldom mutated in neuroblastoma, but several mecha- nisms cooperate to its functional inactivation in this tumor. Increased MDM2 levels, due to genetic amplification or constitutive inhibition of p14ARF, significantly contribute to this event highlighting p53 reactivation as an attractive perspective for neuroblastoma treat- ment. In addition to its role in tumorigenesis, MYCN sensitizes untransformed and cancer cells to apoptosis. This is associated to a fine modulation of the MDM2-p53 pathway Indeed MYCN induces p53 and MDM2 transcription, and, by evoking a DNA damage response (DDR), it stabilizes p53 and its proapoptotic kinase Homeodomain Interacting Prote…