Neuroblastoma: biology and molecular and chromosomal pathology

• Published in: The lancet oncology Vol. 4; no. 8; pp. 472 - 480
• Main Authors: Schwab, Manfred, Westermann, Frank, Hero, Barbara, Berthold, Frank
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01.08.2003; Elsevier Limited
• Subjects: Chemotherapy; Child; Gene Amplification; Gene Dosage; Humans; Loss of Heterozygosity; Medical prognosis; Metastasis; Neuroblastoma; Neuroblastoma - diagnosis; Neuroblastoma - genetics; Neuroblastoma - physiopathology; Pathology; Patients; Receptors, Nerve Growth Factor - physiology; Tumors
• ISSN: 1470-2045; 1474-5488
• DOI: 10.1016/S1470-2045(03)01166-5

Neuroblastoma is the most frequently occurring solid tumour in children, with an incidence of 1·3 cases per 100 000 children aged 0–14 years. Despite many advances during the past three decades, neuroblastoma has remained an enigmatic challenge to clinical and basic scientists. 20 years ago, the MYCN gene was found to be amplified in neuroblastomas, and research since then has focused on the search for other genetic markers. It has emerged that neuroblastoma cells, like cells of many other tumour types, often suffer from extensive, non-random genetic damage at multiple genetic loci. Elucidation of the exact molecular make-up of neuroblastomas will enable researchers to analyse how much specific markers, alone or in combination, can help to stratify disease in prospective studies; at present, stratification is based on age, stage, MYCN, and Shimada pathology. Neuroblastoma may be one of the first examples of the use of genetic tumour markers as a tool for defining tumour behaviour and to aid clinical staging.