Biology of tumors of the peripheral nervous system

Abstract

Tumors of the peripheral nervous system include neuroblastomas, pheochromocytomas, and neuroepitheliomas. Neuroblastomas and pheochromocytomas are adrenergic in origin and share certain genetic features, whereas neuroepitheliomas are thought to be cholinergic and are characterized by distinct genetic features. Neuroblastomas are characterized by deletion of the short arm of chromosome 1 (1p), amplification of the MYCN proto-oncogene, and hyperdiploidy in subsets of tumors. All three of these genetic features have prognostic value in subsets of patients. Allelic loss of 14q also occurs with increased frequency, but the prognostic importance of this abnormality is not known yet. Pheochromocytomas have not been studied as extensively, but allelic loss for 1p appears to be a frequent change, and no clear examples of oncogene activation have been identified to date. Neuroepitheliomas are characterized by translocation between chromosomes 11 and 22. Although they have a characteristic pattern of proto-oncogene expression, it is not clear that any of these oncogenes are activated specifically, and no sites of allelic loss have been identified to date. Thus, cytogenetic and molecular analysis of neuroblastomas, pheochromocytomas, and neuroepitheliomas are useful in distinguishing them from each other and from other tumors in selected cases. Furthermore, certain genetic markers are useful in predicting clinical behavior, especially for neuroblastoma.