Genomic medicine and neuroblastoma.
Neuroblastic tumors are a broad biological and clinical spectrum of neoplastic disease that has long captured the attention of clinicians and scientists alike. It is the most common solid extracranial tumor in children and accounts for 8-10% of all childhood tumors. Tumors are derived from neural crest cells and neural differentiation is common. Neuroblastoma is unique in that it presents with at least three distinct patterns of disease. Locoregional disease (Stage 1, 2, 3) does not metastasize to bone or bone marrow. Stage 4 is a systemic disease with widespread metastasis that responds to chemotherapy but many develop resistance. Stage 4s presents in infancy, is widespread and can spontaneously regress with no intervention, leaving a focus of fibrosis or calcification. Prognosis correlates with age, stage and tumor biological profile. The goal of this review is to provide an overview of the disease and highlight diagnostic, prognostic and therapeutic advances in neuroblastoma. Recommendations and resources for the evaluation and treatment of this disease are outlined.
Oppenheimer
Orit
O
Departments of Pathology and Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
Alaminos
Miguel
M
Gerald
William L
WL
D014408:Biomarkers, Tumor; D002869:Chromosome Aberrations; D005821:Genetic Techniques; D006801:Humans; D009154:Mutation; D009447:Neuroblastoma / Q000175:diagnosis / Q000235:genetics* / Q000378:metabolism / Q000473:pathology
D014408:Biomarkers, Tumor