Abstract
Neuroblastoma (NB) is the most common extracranial embryonic tumor in children with a variety of molecular biological and clinical characteristics. There is no single molecular genetic mechanism involved in the pathogenesis of NB, which determines its heterogeneity. Pathogenetically important event in the development of NB are aberrations of ALK gene (Anaplastic lymphoma kinase), which are found in 70 % of patients with familial form of NB and in 7– 10 % of patients with sporadic cases. ALK oncogene encodes a receptor of the same name, expressed on the membrane of cells of the central and peripheral nervous system, which is in the activated state in NB. The negative effect of ALK gene anomalies on the prognosis in patients with different risk groups of NB is described. ALK gene aberrations are more often detected duringrelapse and refractory course of the disease. Because of its tissue specificity, ALK protein is an ideal target for targeted therapy. This article presents a literature review of the role of ALK in NB.
Highlights
There is no single molecular genetic mechanism involved in the pathogenesis of NB, which determines its heterogeneity
Important event in the development of NB are aberrations of ALK gene (Anaplastic lymphoma kinase), which are found in 70 % of patients with familial form of NB and in 7–10 % of patients with sporadic cases
ALK oncogene encodes a receptor of the same name, expressed on the membrane of cells of the central and peripheral nervous system, which is in the activated state in NB
Summary
Information about the authors N.A. Andreeva: Pediatric Oncologist, Department of Clinical Oncology Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia, e-mail: andreeva793@bk.ru; https://orcid.org/0000-0001-5626-218X A.E. Druy: Cand. (Med.), Senior Researcher, Laboratory of Cytogenetics and Molecular Genetics Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia, e-mail: Dr-Drui@yandex.ru; https://orcid.org/0000-0003-1308-8622 T.V. Shamanskaya: Cand.
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