Abstract

ALVAREZ, ANDRÉS M.  and  LAMARRE, REYNALD. STATE OF ART: NEURAL CREST TUMORS, NEUROBLASTOMA (NB) PATHOPHYSIOLOGY, DIAGNOSIS AND TREATMENT. rev.fac.med [online]. 2012, vol.20, n.2, pp.38-49. ISSN 0121-5256.

Neuroblastoma (NB) is the most common extracranial solid cancer in childhood and the most common cancer in infancy, with an annual incidence of about 650 cases per year in the US, and 100 cases per year in Italy and Spain. Roughly 50 percent of neuroblastoma cases occur in children younger than two years of age. It is a neuroendocrine tumor, arising from neural crest cells of the sympathetic nervous system (SNS). It most frequently originates in one of the adrenal glands, but can also develop in nerve tissues located in the neck, chest, abdomen, and pelvis. It is a highly variable tumor that can spontaneously regress or have highly malignant behavior, especially in older children with disseminated disease. Signs and symptoms of neuroblastoma reflect the presentation of tumor location and because of the multiple clinical presentations, can be confused with a variety of pathologies. Treatment modalities include surgery, chemotherapy, radiotherapy and immunotherapy. The role of each method is determined in advance of the clinical behavior of the tumor on a case specific basis, consideration of the age, stage and biological parameters. Over the past decade, stratification of patients with risk factors for neuroblastoma based on the analysis of a large panel of clinical and biological variables, among which are age, stage and N-MYC amplification, has allowed for early diagnosis and the generation of new guidelines for major advancements in treatment. Today, treatment of neuroblastoma has become increasingly sophisticated and is influenced by risk factors as well as clinical and biological parameters. A better understanding of the biology of neuroblastoma has allowed for the differentiation between tumors that can be cured with minimal treatment and those requiring a multidisciplinary and complex approach in order to have a chance of cure. Early diagnosis with staging is essential in the development of an appropriate therapy.

Keywords : Neuroblastoma; neural crest; brain tumor; childhood; N-MYC; bone; Homer syndrome; NCMAM.

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