Management of Neuroblastoma: ICMR Consensus Document

AbstractNeuroblastoma (NBL) is the most common extra-cranial solid tumor in childhood. High-risk NBL is considered challenging and has one of the least favourable outcomes amongst pediatric cancers. Primary tumor can arise anywhere along the sympathetic chain. Advanced disease at presentation is common. Diagnosis is established by tumor biopsy and elevated urinary catecholamines. Staging is performed using bone marrow and mIBG scan (FDG-PET/bone scan if mIBG unavailable or non-avid). Age, stage, histopathological grading,MYCN amplification and 11q aberration are important prognostic factors utilized in risk stratification. Low-risk disease including Stage 1 and asymptomatic Stage 2 disease has an excellent prognosis with non-mutilating surgery alone. Perinatal adrenal neuroblastoma may be managed with close observation alone. Intermediate-risk disease consisting largely of unresectable/symptomatic Stage 2/3 disease and infants with Stage 4 disease has good outcome with few cycles of chemotherapy followed by surgical resection. Paraspinal neuroblastomas with cord compression are treated emergently, typically with upfront chemotherapy. Asymptomatic Stage 4S disease may be followed closely without treatment. Organ dysfunction and age below 3 mo would warrant chemotherapy in 4S. High-risk disease includes older children with Stage 4 disease andMYCN amplified tumors. High-risk disease has a suboptimal outcome, though the survival is improving with multimodality therapy including a...
Source: Indian Journal of Pediatrics - Category: Pediatrics Source Type: research

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(Uppsala University) Using a computer algorithm, scientists at Uppsala University have identified a promising new treatment for neuroblastoma. This form of cancer in children, which occurs in specialised nerve cells in the sympathetic nervous system, may be life-threatening. In the long term the discovery, described in the latest issue of the scientific journal Nature Communications, may result in a new form of treatment for children in whom the disease is severe or at an advanced stage.
Source: EurekAlert! - Cancer - Category: Cancer & Oncology Source Type: news
Authors: Larsson K, Kock A, Kogner P, Jakobsson PJ Abstract The importance of prostaglandin E2 in cancer progression is well established, but research on its role in cancer has so far mostly been focused on epithelial cancer in adults while the knowledge about the contribution of prostaglandin E2 to childhood malignancies is limited. Neuroblastoma, an extracranial solid tumor of the sympathetic nervous system, mainly affects young children. Patients with tumors classified as high-risk have poor survival despite receiving intensive treatment, illustrating a need for new treatments complimenting existing ones. The ba...
Source: Advances in Experimental Medicine and Biology - Category: Research Tags: Adv Exp Med Biol Source Type: research
In this study, we investigated the impact of the human ALK-F1174L mutation in vivo on sympathetic neuroblast differentiation using Sox10-Cre;LSL-ALK-F1174L mice. The aim was to improve our understanding of the implication of ALK deregulated signaling in the initial steps of NB genesis. Results ALK-F1174L Causes Embryonic Lethality When Expressed in NCCs Prior to SA Lineage Commitment To assess the impact of the human ALK-F1174L activating mutation on the development and differentiation of the SNS, Sox10-Cre;LSL-ALK-F1174L embryos were generated. This model allowed to restrict the expression of the ALK-F1174L variant to ...
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
Publication date: Available online 27 February 2019Source: Radiation Physics and ChemistryAuthor(s): G.C. Ferreira, D.F. Oliveira, R.G. Leitão, J.E.P. Rosa, C. Palmero, E.G. Oliveira-Barros, M.A. Oliveira, L.E. Nasciutti, R.T. Lopes, M.J. AnjosAbstractNeuroblastoma (NB) is a malignancy of the sympathetic nervous system that almost exclusively occurs during infancy and early childhood. NB is responsible for 8 to 10% of all childhood cancers. More than 50% of the diagnosed cases have metastatic disease at the time of diagnosis. Trace elements are extremely important in biological processes, including reactions involvi...
Source: Radiation Physics and Chemistry - Category: Physics Source Type: research
Authors: Alharris E, Singh NP, Nagarkatti PS, Nagarkatti M Abstract Neuroblastoma (NBL) is one of the most common childhood cancers that originate from the immature nerve cells of the sympathetic system. Studies with NBL cancers have also shown that miRNAs are dysregulated and may play a critical role in pathogenesis. Cannabidiol (CBD) is a non-psychoactive compound found in marijuana which has been previously shown by our laboratory and others to induce apoptosis in cancer cells. However, there are no studies reported to test if CBD mediates these effects through regulation of miRNA. In the current study, therefor...
Source: Oncotarget - Category: Cancer & Oncology Tags: Oncotarget Source Type: research
Authors: Ognibene M, Pagnan G, Marimpietri D, Cangelosi D, Cilli M, Benedetti MC, Boldrini R, Garaventa A, Frassoni F, Eva A, Varesio L, Pistoia V, Pezzolo A Abstract Neuroblastoma is an aggressive, relapse-prone childhood tumor of the sympathetic nervous system that accounts for 15% of pediatric cancer deaths. A distal portion of human chromosome 3p is often deleted in neuroblastoma, this region may contain one or more putative tumor suppressor genes. A 2.54 Mb region at 3p26.3 encompassing the smallest region of deletion pinpointed CHL1 gene, the locus for neuronal cell adhesion molecule close homolog of L1. We f...
Source: Oncotarget - Category: Cancer & Oncology Tags: Oncotarget Source Type: research
Neuroblastoma (NB) is an early childhood malignancy that arises from the developing sympathetic nervous system. Harmine is a tricyclic β-carboline alkaloid isolated from the harmal plant that exhibits both cyt...
Source: Cancer Cell International - Category: Cancer & Oncology Authors: Tags: Primary research Source Type: research
Abstract Neuroblastoma (NB) is a tumor of the sympathoadrenal system arising in children under 15 years of age. In Germany, NB accounts for 7% of childhood cancer cases, but 11% of cancer deaths. It originates from highly migratory progenitor cells that leave the dorsal neural tube and contribute neurons and glial cells to sympathetic ganglia, and chromaffin and supportive cells to the adrenal medulla and paraganglia. Clinically, histologically and molecularly, NBs present as extremely heterogeneous, ranging from very good to very poor prognosis. The etiology of NB still remains unclear and needs to be elucid...
Source: Cellular and Molecular Life Sciences : CMLS - Category: Cytology Authors: Tags: Cell Mol Life Sci Source Type: research
Neuroblastoma is a childhood cancer that is fatal in almost half of patients despite intense multimodality treatment. This cancer is derived from neuroendocrine tissue located in the sympathetic nervous system. Glypican-2 (GPC2) is a cell surface heparan sulfate proteoglycan that is important for neuronal cell adhesion and neurite outgrowth. In...
Source: Proceedings of the National Academy of Sciences - Category: Science Authors: Tags: PNAS Plus Source Type: research
ur GM Abstract Retinoblastoma (RB) is the most common intraocular malignancy in childhood. Approximately 40% of retinoblastomas are hereditary and due to germline mutations in the RB1 gene. Children with hereditary RB are also at risk for developing a midline intracranial tumor, most commonly pineoblastoma. We recommend intensive ocular screening for patients with germline RB1 mutations for retinoblastoma as well as neuroimaging for pineoblastoma surveillance. There is an approximately 20% risk of developing second primary cancers among individuals with hereditary RB, higher among those who received radiotherapy f...
Source: Clinical Cancer Research - Category: Cancer & Oncology Authors: Tags: Clin Cancer Res Source Type: research
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