Researchers at the Sahlgrenska Academy at the University of Gothenburg, Sweden and Karolinska Institutet have used novel technology to reveal the different genetic patterns of neuroblastoma, an aggressive form of childhood cancer. This discovery may lead to significant advances in the treatment of this malignant disease, which mainly affects small children.
The article is being published in the Proceedings of the National Academy of Sciences (PNAS). The study includes 165 children with neuroblastoma, most of whom developed the disease before the age of five. These children have been monitored for over 20 years by two research teams led by professors Tommy Martinsson, of the Sahlgrenska Academy, and Per Kogner of Karolinska Institutet.
Neuroblastoma is a nerve cell cancer that has defects in certain chromosomes. If the tumour has a characteristic defect on chromosome 11, it is very aggressive and difficult to cure.
"We found that the children who develop this type of neuroblastoma are twice as old at the onset of the disease as children who develop other types of neuroblastoma. This type progresses more slowly and is more difficult to treat," says Helena Carén, a researcher at the Department of Clinical Genetics at the Sahlgrenska Academy.
By using the latest genetic techniques, the researchers have succeeded in analysing the DNA of tumour cells and identifying chromosomal defects, enabling the identification of sub-groups of the most aggressive neuroblastomas. The next step is to identify their weak points genetically in order to develop better treatment.
"We call this personalized medicine, because the treatment is based on the genetic profile of the patient, or in this case, of the tumour cells," says Tommy Martinsson, professor of genetics at the Department of Clinical Genetics at the Sahlgrenska Academy.
Per Kogner, professor of paediatric oncology at Karolinska Institutet, reiterates that their discovery will now allow a variety of tailor-made treatments to be developed, saving the lives of more children.
"The analytical method we have used in our research is already being used for clinical assessment of every neuroblastoma tumour in the country, which means that we can now make more accurate diagnoses," says Helena Carén.
The study was carried out with the support of the Swedish Childhood Cancer Foundation and the Swedish Cancer Society.
Neuroblastoma is a form of cancer that affects small children, most of whom are diagnosed before they reach their fifth birthday. It is the third commonest form of cancer in children, after leukaemia and brain tumours. About 20 Swedish children are affected every year, and the risk of developing the disease is the same worldwide. Neuroblastoma is a tumour of nerve cells. It appears during the development phase of the sympathetic nervous system. Children may have no symptoms at all, and sometimes a lump is the first sign of the disease noticed by parents or doctors. As the tumour grows or spreads, it may press on other organs and cause symptoms. The available treatments include surgery, chemotherapy, radiotherapy, high-dose therapy combined with stem cell support, and vitamin A.
- Helena Carén, Hanna Kryh, Maria Nethander, Rose-Marie Sjöberg, Catarina Träger, Staffan Nilsson, Jonas. High-risk neuroblastoma tumors with 11q-deletion display a poor prognostic, chromosome instability phenotype with later onset. Proceedings of the National Academy of Sciences, 2010; DOI: 10.1073/pnas.0910684107
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