Recently a publication came out in the journal Child's Nervous System retrospectively reviewing patient characteristics and outcomes in children treated for DIPG over a 13 year period (February 1999 to May 2012) at Cerrahpassa Medical Faculty and Oncology Institute in Instanbul, Turkey.
In this review there were 26 girls and 24 boys with a median age of 7. The median duration of symptoms - including cranial nerve palsies, motor disability and/or cerebellar dysfunction- was 30 days. The diagnosis was made by a multidisciplinary tumor board which included a a pediatric oncologist, radiation oncologist, neurosurgeon, and radiologist in the multidisciplinary tumor board.
Radiation was part of all the children's therapy although only twelve received radiation alone. The other children also received some form of chemotherapy with radiation. In 17 patients a radiosensitizer (either vincristine or cisplatin) during radiation and followed by CCNU and vincristine after radiation. After temozolomide became available this agent was used both during and after radiation for the remaining 21 children.
These three groups were analyzed regarding outcomes: group 1-radiation alone; group 2-vincristine/ciplastin; group 3- temozolomide. In this study, children in either chemotherapy group did better than those that had radiation alone. Ten of the children in group 2 were alive at 2 years and 3 at 3 years. Three children in group 3 were also alive at 3 years.
Interestingly 3 children in the temozolomide group had biopsies and were found to have pilocytic astroctyomas!
Fo me it is hard to know exactly what caused these two chemotherapy groups had better survival. It would seem that the 3 children with pilocytic astrocytomas were included in the analysis. The authors also said that there were improvements in radiation techniques over time which could have been a factor. Thus children who had radiation alone were the earliest patients in the review. Also it seems over type palliative care, PEG tubes and shunts became more common in that institution which may have played a role in the statistics.
Positive results of temozolomide with DIPG have not been replicated in other countries. Still, this article has significance to me to show the increase interest in DIPG around the world as well as the improved treatment for children with cancer. It was also good to see the conclusion that "the complex biology of DIPG renders an unselected single-agent approach less likely to be effective. Instead, a multi-targeted approach seems to be required to improve the prognosis". Hopefully we will see increasingly available multi-targeted options for children with DIPG.
Reference:
Pediatric diffuse intrinsic pontine glioma patients from a single center
Childs Nerv Syst. 2013 Apr;29(4):583-8. doi: 10.1007/s00381-012-1986-3. Epub 2012 Dec 8.
http://www.ncbi.nlm.nih.gov/pubmed/23224361
Radiotherapy with concurrent and adjuvant temozolomide in children with newly diagnosed diffuse intrinsic pontine glioma (France)
http://www.ncbi.nlm.nih.gov/pubmed/21858607
Temozolomide in the treatment of children with newly diagnosed diffuse intrinsic pontine gliomas: a report from the Children's Oncology Group
http://www.ncbi.nlm.nih.gov/pubmed/21345842
Prospective evaluation of radiotherapy with concurrent and adjuvant temozolomide in children with newly diagnosed diffuse intrinsic pontine glioma (India)
http://www.ncbi.nlm.nih.gov/pubmed/19647954
