Researchers at Perth's Telethon Institute for Child Health Research have found new genetic differences in one of the rarest and most aggressive types of children's cancer that may present new opportunities in treating the disease, NUT-midline carcinoma (NMC).
Details of the analysis have been released online ahead of publication in leading international cancer journal Oncogene.
NMC usually affects organs along the midline of the body. The first case was identified by the Head of the Telethon Institute's Children's Leukaemia and Cancer Research Laboratory, Professor Ursula Kees, in 1991.
Researchers Dr Alex Beesley and Professor Kees said that 63 cases with NMC have now been recorded worldwide, mostly in children. While extremely rare, the cancer is very aggressive and progresses rapidly.
"Current treatments are ineffective and, to date, only one patient in the world has survived," Dr Beesley said.
"Our research is trying to identify the Achilles heel of these types of tumour so we can develop better therapeutic strategies."
"In this study, we have found a very strong genetic feature in these tumours in the form of a gene fusion. It is unusual to have such a strong single feature and this could be an important step in targeting treatments."
There are very few established cell-lines of NMC for research in the world. Of those, three have been grown by the research team at the Telethon Institute.
Dr Beesley said research into this rare cancer had been made possible by funding from the Perth-based Children's Leukaemia and Cancer Research Foundation (CLCRF).
"Our next step is to test a range of existing drugs against this particular genetic target. At the moment, clinicians have no effective treatments for NMC and it's crucial that we find something that can slow or stop this terrible disease."
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Citation: Novel BRD4-NUT fusion isoforms increase the pathogenic complexity in NUT midline carcinoma. Thompson-Wicking K, Francis RW, Stirnweiss A, Ferrari E, Welch MD, Baker E, Murch AR, Gout AM, Carter KW, Charles AK, Phillips MB, Kees UR, Beesley AH. Oncogene. 2012 Nov 5. doi: 10.1038/onc.2012.487. [Epub ahead of print] PMID: 23128391 [PubMed - as supplied by publisher]