11q deletion in neuroblastoma: a review of biological and clinical implications

Abstract

Deletion of the long arm of chromosome 11 (11q deletion) is one of the most frequent events that occur during the development of aggressive neuroblastoma. Clinically, 11q deletion is associated with higher disease stage and decreased survival probability. During the last 25 years, extensive efforts have been invested to identify the precise frequency of 11q aberrations in neuroblastoma, the recurrently involved genes, and to understand the molecular mechanisms of 11q deletion, but definitive answers are still unclear. In this review, it is our intent to compile and review the evidence acquired to date on 11q deletion in neuroblastoma.

Keywords: 11q deletion; Cancer; Development; MYCN amplification; Metastasis; Neuroblastoma; Review.

Fig. 1

Molecular networks between genes involved in NB development by the data mining IPA software. Nine genes (red and green) on 11q recognized in regulatory networks involving MYCN, MAPK, PGE2, ATM and p53 (experimentally described interaction only). All genes presented in this figure are highly associated with cancer (27/36 molecules, p-value = 2.41E-18). Where appropriate, proteins (white) were added by IPA software to complete the pathway. Legend contains description of basic protein functions, nature of interaction and relation to commonly mutated genes in NB or 11q deletion [10, 134]

Fig. 2

Location of deletions on 11q arm in NB tumors. a Location of deletions on 11q arm in NB tumors. b Copy number segmentation plot of chromosome 11 of 214 non-MNA high-risk tumors; horizontal lines represent single tumor dosages represented as the base 2 logarithm (y axis) of the copy number along the chromosome; vertical lines indicate breakpoints associated with gain (red) and loss (blue) of genomic regions. c Analogously to b, segmentation plot of chromosome 11 in 103 MNA tumors