Neuroblastoma and other childhood neural tumors: a review

Neuroblastoma is the most common nonhematopoietic solid tumor of childhood and has been intensively studied for at least 4 decades. Despite this, few predictive histopathologic clues to its behavior exist. Age, anatomic sites of occurrence, and clinical stage have traditionally been the only reliable prognostic factors in this disease. A number of laboratory studies that focus on biologic features such as neurotransmitter synthesis (adrenergic and noradrenergic catecholamines), neurotransmitter enzyme expression (dopamine beta hydroxylase, choline acetyl transferase), cytogenetics (homogeneously staining regions, double minute chromosomes, chromosome 1p deletions), molecular genetics (N-myc oncogene amplification and expression), and immunophenotype (surface epitopes such as HLA antigens and GD2 ganglioside and intracytoplasmic determinants such as neurofilament protein, synaptophysin, chromogranin, and neuron specific enolase) now enable the pathologist to predict clinical course in many cases and to distinguish bona fide neuroblastomas, regardless of age, site, or histologic appearance, from a host of related but distinctly separate neuroectodermal tumor entities with apparent different histogenesis, treatment sensitivity, and prognosis.