Swedish researchers share the prize at SIOP for winning posters on neuroblastoma
Drs Fredrik Hedborg (Uppsala University) and Catarina Trägar (Karolinska Institute) shared the top prize for neuroblastoma research poster at SIOP 2010.
PH036 Age dependent genotypes in high-risk neuroblastoma: MYCN amplification is a fast track to aggressive disease whereas segmental deletion of 11q implies a more complex, multi-step tumor evolution (p. 896)
Dr Hedborg and colleagues explored the age-dependence of the genetics of aggressive disease in 30 high-risk and 4 intermediate-risk children. MYCN amplification was present in all but one of the 12 youngest children (mean age 29.6 months, range 4 – 30 months) and MYCN amplification was absent in all but one of the 11 oldest (mean age 65.6 months, range 57 – 169 months), and 12/18 of these had 11q loss. This age differential was confirmed by the Swedish Childhood Cancer Registry where mean ages at diagnosis were 29.4 months for MYCN amplified (n=65) and 54.8 months for MYCN-non-amplified (n=46). Significantly more segmental chromosome aberrations were noted in older children with 11q loss, and this data suggest that two major pathways exist in the development of aggressive neuroblastoma. MYCN-amplification tumors in younger children result from fewer but rapid genetic events, whereas tumors in older children with 11q loss are the result of a slower, multi-step process.
PH038 Differences in biological features and survival improvement between genetic subsets of high-risk neuroblastoma indicate the need of adapted treatment (p. 897)
Dr Trägar and colleagues also looked at Swedish registry data and noted older age for 34 children with 11q deletion (median age 41 months) and longer median survival of 40 months compared to children with MYCN-amplified tumors (median age 22.5 months, median survival 16 months), but both groups had similar 8 year overall survival rate of ~35%.
Survival data were reported as follows:
|dates||5-yr OS for all NB risk groups|
|1982 – 1990||57.7%|
|2000 – 2009||78.6%|
|dates||High-risk NB||High-risk NB
|1982 – 1990||n = 36||11.1%|
|1991 – 1999||n = 56||17.9%|
|2000 – 2009||n = 56||61.6%|
|11q loss ,
|1982 – 1995||11.1||37.5%|
|1996 – 2009||48.9%||42.9%|
The researchers concluded that 11q-deletion tumors present later than MYCN-amplified tumors, but noted that prognosis is similar, and suggest further consideration is needed for therapeutic approaches for 11q-deleted tumors since prognosis has not improved for this group since the 1980s.
Both research teams have contributed to increased understanding concerning the relationship between age and biology of neuroblastoma high-risk tumors.
Travel to this meeting was supported by:
- Lighthouse Laboratories
- Max’s Ring of Fire
- Magic Water
- Friends of Will
- Solving Kids’ Cancer
- Children’s Neuroblastoma Cancer Foundation