Format

Send to:

Choose Destination
See comment in PubMed Commons below
Cancer Lett. 2003 Jul 18;197(1-2):41-5.

Familial neuroblastoma: a complex heritable disease.

Author information

  • 1Laboratory of Tumor Genetics, Istituto Nazionale per la Ricerca sul Cancro (IST), 16132 Genoa, Italy.

Abstract

Genomic surveys carried out over the years have revealed a great heterogeneity in the pattern of genetic aberrations occurring in neuroblastoma (NB) cells. Studies on familial NB could lead to the discovery of susceptibility genes and their contribution to the development of the disease. The two-hit hypothesis is considered the most consistent model of inherited predisposition to NB, but an oligogenic inheritance governed by a major gene should be also taken into account. The rarity of familial clustering of NB cases and the difficulty to recruit informative families has not allowed the identification of the disease gene until now. In fact, linkage analysis has suggested more than one region candidate to harboring NB predisposing gene(s). These findings indicate that genetic heterogeneity might be the molecular basis of the disorder. Given the complexity of the disease additional studies are required to elucidate the genetic determination of NB.

PMID:
12880958
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science
    Loading ...
    Write to the Help Desk