Neuroblastoma

Abstract

Neuroblastoma is a heterogeneous disease; tumors can spontaneously regress or mature, or display an aggressive, therapy-resistant phenotype. Increasing evidence indicates that the biologic and molecular features of neuroblastoma significantly influence and are highly predictive of clinical behavior. Because of this, neuroblastoma has served as a paradigm for biological risk assessment and treatment assignment. Most current clinical studies of neuroblastoma base therapy and its intensity on a risk stratification that takes into account both clinical and biologic variables predictive of relapse. For example, surgery alone offers definitive therapy with excellent outcome for patients with low-risk disease, while patients at high-risk for disease relapse are treated with intensive multimodality therapy. In this review recent advances in the understanding of the molecular genetic events involved in neuroblastoma pathogenesis are discussed, and how they are impacting the current risk stratification and providing potential targets for new therapeutic approaches for children with neuroblastoma. In addition, the results of significant recent clinical trials for the treatment of neuroblastoma are reviewed.

β-Arrestin1 and Distinct CXCR4 Structures Are Required for Stromal Derived Factor-1 to Downregulate CXCR4 Cell-Surface Levels in Neuroblastoma

“…Neuroblastoma is the most common malignancy in infants, causing one case in every 100,000 children in the United States (Verissimo et al, 2011;Davidoff, 2012). High-risk neuroblastoma patients undergo intensive chemotherapy, stem cell harvesting, and primary tumor resection (Davidoff, 2012), interventions that improve prognosis but cause significant morbidity, developmental delays, leukemia risk, and infertility.…”

“…High-risk neuroblastoma patients undergo intensive chemotherapy, stem cell harvesting, and primary tumor resection (Davidoff, 2012), interventions that improve prognosis but cause significant morbidity, developmental delays, leukemia risk, and infertility. There is an urgent need for neuroblastoma treatments that spare normal tissue, including normal developing hematopoietic and other stem cells.…”

Ganglioneuroblastoma in an adult patient

“…[1,3] NB and GNB are often grouped together because both possess malignant potential. [3] NB is a tumor composed of immature undifferentiated neuroblasts while GNBs are comprised of both mature and immature cells.…”

“…It comprises about 8% of all pediatric cancers and accounts for approximately 15% of all cancer deaths in patients under the age of 15. [1][2][3] However, it is rare in adults and therefore most research and literature pertains to NB in children. There is a limited number of cases of NB reported in adults.…”

N-Myc Inhibition: Advances in Neuroblastoma Treatment

“…Neuroblastoma (NBL), the most common solid tumor of childhood, is originated from the neural crest cells of the developing sympathetic nervous system [1]. It represents 8% -10% of pediatric tumors and accounts for 15% of all pediatric cancer deaths [2] [3] [4].…”