What is it about?
Neuroblastoma probably derives from primitive sympathetic neural precursor cells of the peripheral nervous system. The most common primary tumour site of neuroblastoma is adrenal (47%), followed by abdominal/retroperitoneal (24%), thoracic (15%), pelvic (3%) and neck (3%). This article reviews the biological/genetic features and their clinical importance in neuroblastoma.
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Why is it important?
Neuroblastoma is the most common embryonic malignancy diagnosed during the first year of life. In addition, it is the most common extracranial solid tumour in High-income Countries (HIC), and it accounts for 6-9.7% (ratio of annual rates per million population) of all cancers observed in children younger than 15 years of age. To explore molecular mechanisms underlying the cellular and clinical behaviours occurring throughout the progression of neuroblastoma, this review focused on the chromosomal abnormalities and cellular signalling pathways involving apoptosis, differentiation, spontaneous regression, proliferation and metastasis.
Perspectives
Neuroblastoma is a complex disease that has different clinical courses, from metastatic spread to spontaneous regression. It is a heterogeneous tumour that can show many different chromosomal abnormalities. The clinical, biological, genetic and biochemical factors such as age ≥ 18 months, MYCN amplification, 1p deletion, 17q gain, 11q LOH, diploidy, TrkB/BDNF expression, and high LDH and ferritin levels are associated with worse prognosis in neuroblastoma. On the other side, NGF/TrkA signalling, various other signalling pathways involving apoptosis, differentiation and impaired DNA repair, telomere shortening, adaptive T cell-mediated immunity and epigenetic control contribute to spontaneous regression of neuroblastoma. I think that these biological, genetic and clinical features are necessary to be evaluated together to develop new treatment strategies for neuroblastoma patients.
Nevim Aygün
Read the Original
This page is a summary of: Biological and Genetic Features of Neuroblastoma and Their Clinical Importance, Current Pediatric Reviews, August 2018, Bentham Science Publishers,
DOI: 10.2174/1573396314666180129101627.
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