Pathogenesis and treatment of neuroblastoma

Abstract

Neuroblastoma (NB) is the most common extracranial solid tumor that occurs in children. NB is highly metastatic and varies in risk level according to tumor histology and genetic characteristics. The amplification of the MYCN oncogene is one of the key factors that promotes the development of tumors and is associated with poor prognosis. Mutations in the tyrosine kinase ALK gene have also been linked to tumorigenesis, especially in familial neuroblastoma. Other genetic mutations that have been identified include ATRX, PTPN11, ARID1A, ARID1B, and genes involved in neuritogenesis. Genetic aberrations like chromothripsis, the loss of chromosome segments 1p and 11q, as well as the gain of segment 17q may also be linked to the development of NB. Treatment for this disease varies according to the patient’s risk profile but may include surgery, high dose chemotherapy with or without bone marrow transplantation, and radiation. Several immunotherapy drugs, as well as drugs that target the disease pathway, are currently in development with the goal of improving treatment, survival rates, and quality of life. This review will discuss the molecular and genetic factors that drive neuroblastoma. It will also discuss the current treatments and survival rates of the disease as well as recent and ongoing treatment research.