Journal Watch

Sick Kids is becoming a powerhouse for diffuse intrinsic pontine glioma research.
This past February, Sick Kids hosted an International Think Tank with approximately 45 researchers, clinicians and advocates to specifically address issues regarding this tumor. In February, this same team published the first whole genomic profiling of DIPG tumors highlighting platelet derived growth factor receptor alpha as a potential target for therapy. In the May issue of the Journal of Neuro-oncology, the same team published their six year experience with post-mortem examination of diffuse intrinsic pontine gliomas.

Between July 2002 and June 2008, 27 children were diagnosed by a combination of clinical symptoms and radiographic appearance to have a diffuse intrinsic pontine glioma. The clinical symptoms included cranial nerve palsies, ataxia and long tract signs lasting less 6 months. The radiographic criteria included that the mass was at least 2/3 of the pons without an exophytic component.
Here are some of the statistics that were presented regarding these 27 children

• There was an even gender split- 13 male and 14 female.
• The median age at diagnosis was 7.1 years (range - 0 days to 16.9 years) .
• Twenty- four children had radiation. The three that did not have radiation were of a very young age.
• Sixty three percent of the children had a partial or complete clinical response to radiation.
• The media time to progression at the end of radiation was 3.9 months (range – 0.8 to 13. 6 months).
• The median time from diagnosis to death was 10.1 months (range – 0.3- 26.3 months).

Autopsy tumor donation was discussed with 21 of the families. Ten consented. Regarding the six families where this was not discussed- 4 children had transferred care to another provider and with two families the physicians felt that the family was too overwhelmed.

Due to logistical issues only nine of the ten consented donations were obtained. All specimens were adequate for nucleic acid. Interestingly, two of the nine samples were found to be primitive neurectodermal tumors (PNETs). The other seven were glioblastomas.



Reference:

J Neurooncol. 2010 May 16
Post mortem Examination in diffuse intrinsic pontine glioma: challaneges and chances.
Angelini P, Hawkins, C, Laperriere N, Bouffet E, Bartels U.
Division of Paediatric Haematology and Oncology, The Hospital for Sick Children, Toronto, Canada