Children's cancer targeted by ARC grant

The survival rate of children with childhood cancers has increased spectacularly since the early 1960s, from a bleak 10 per cent to around 75 per cent today. One notable exception to the trend is neuroblastoma, with a survival rate below 50 per cent.

Assoc Prof Murray Norris, deputy director of the Children's Cancer Institute Australia (CCIA) in Sydney, says neuroblastoma develops in the precursor cells that form the sympathetic nervous system. The tumour is commonly present, but undetectable, at birth, usually in the abdominal region.

By the time most infants are diagnosed with neuroblastoma, the primary tumour has metastasised, spawning secondary tumours in vital organs. Norris says that, after leukaemia, it is the most common cause of death among young children in developed nations.

Tumours tend to develop in the abdomen, usually above a kidney, and Norris says they usually respond poorly to chemotherapy and radiation therapy.

Little is known about what causes neuroblastoma. Many childhood cancers have a strong hereditary component, but with no clear familial pattern evident in neuroblastoma, environmental factors may be involved. Norris said some studies have implicated in utero exposure to carcinogens in tobacco smoke as a risk factor.

The well known oncogene c-Myc is amplified in tumour cells in 25 to 30 per cent of young patients. "These patients do particularly poorly," Norris said.

Over-expression of the human c-MYC oncogene in neural cells causes neuroblastoma-type tumours in a transgenic mouse model of the disease.

Norris and two colleagues -- CCIA director Prof Michelle Haber and Prof Andre Gudkov, of the Cleveland Clinic Foundation in Cleveland, Ohio -- have received an Australian Research Council (ARC) Linkage Grant of just over $200,000 over three years to investigate neuroblastoma and develop a high-throughput, small molecule drug-screening program.

The commercial partner in the study is Quark Biotech, based in Israel.

Norris says the study will apply two novel functional genomics techniques developed by Gudkov to identify key genes involved in neuroblastoma.