Genetic insights into familial tumors of the nervous system

Authors

  • German Melean,

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    • Dr. German Melean, M.D., is a fellow in medical genetics at the Medical Genetics Unit, Department of Clinical Physiopathology, University of Florence, Florence, Italy.

  • Roberta Sestini,

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    • Dr. Roberta Sestini, Ph.D., is research assistant at the Medical Genetics Unit, Department of Clinical Physiopathology, University of Florence, Florence, Italy.

  • Franco Ammannati,

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    • Dr. Franco Ammannati, M.D., is a neurosurgeon specialized in the treatment of hereditary nervous system tumors. He is on staff at the Neurosurgery Unit of Careggi Hospital, Florence, Italy.

  • Laura Papi

    Corresponding author
    • Dipartimento di Fisiopatologia Clinica, Sezione di Genetica Medica, Università degli Studi di Firenze, Viale Pieraccini, 6, 50139 Firenze, Italy.
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    • Professor Laura Papi, M.D., Ph.D., is a medical geneticist, Associate Professor of Medical Genetics at the Department of Clinical Physiopathology of the University of Florence, Florence, Italy. She has a long-standing research interest in genetic aspects of inherited cancer syndromes.


Abstract

Nervous system tumors represent unique neoplasms that arise within the central and peripheral nervous system. While the vast majority of nervous system neoplasm occur sporadically, most of the adult and pediatric forms have a hereditary equivalent. In a little over a decade, we have seen a tremendous increase in knowledge of the primary genetic basis of many of the familial cancer syndromes that involve the nervous system, syndromes that are mostly inherited as autosomal dominant traits. In this review, we discuss the most recent findings on the genetic basis of hereditary nervous system tumors. The identification of genes associated with familial cancer syndromes has in some families enabled a “molecular diagnosis” that complements clinical assessment and allows directed cancer surveillance for those individuals determined to be at-risk for disease. © 2004 Wiley-Liss, Inc.

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