Biopsy Consensus Statements on DIPGs

Over the last few years there have been rapid change in treating and understanding pediatric brain tumors.   Per this recently electronically published article in Neuro Oncology these include :
1) Brainstem tumors are now considered to be safe targets for either stereotactic or open biopsy;
2) Age is a major factor interacting with tumor behavior (progression risk);
3) Recent research identifying new biologic markers of tumor classification and new druggable targets for trials has provided and opportunity to explore novel approaches to therapy.

In  February 2011, the second Consensus Conference on Pediatric Neurosurgery (CPN 2011) was held in Paris, France with the objective to establish a new consensus on surgical approaches to pediatric gliomas.   Statements on high grade gliomas, hypothalamic chiasmatic gliomas and diffuse intrinsic pontine gliomas were developed.  Statements  were disseminated to the 92 participants for a vote.  A statement was accepted if there was more than 70% of votes in favor of the statement.

Here are the final statements regarding DIPGs.
Typical DIPG
Biopsy of  a typical DIPG (defined by a short history and typical imaging findings) is justified when the patient is part of an ethically approved clinical study in which the tissue obtained will be used to investigate or inform the role of biological markers after treatment selection or molecular tumor grading.

Atypical Pontine Region Tumors
(A) Biopsy by an experienced pediatric neurosurgeon is indicated to confirm the diagnosis and guide therapy,
(B) An atypical pontine region tumor would be considered separately from classic DIPG for therapy or research purposes.

Authors of the final consensus paper included:
* David A Walker- Nottingham, United Kingdom
* JoFen Liu- Boston, USA
* Mark Kieran- Boston,  USA
* Nada Jabado- Quebec, Canada
* Susan Picton- Leeds, United Kingdom
* Roger Packer- Washington, USA
* Christian St. Rose - Paris, France

References:
A multi-disciplinary consensus statement concerning surgical approaches to low grade, high grade astrocytomas and diffuse intrinsic pontine gliomas (CPN Paris 2011) using the Delphi method.
Neuro Oncol 2013 March 15 [epub ahead of print]
http://www.ncbi.nlm.nih.gov/pubmed/23502427

French DIPG Site- Tumeur du Tronc Cérébral

Prior to the past decade or so it has been virtually impossible to truly connect DIPG famililes as a community in any significant way.   The internet has changed all that.  Not only is there virtually instantaneous communication- but that communication can happen even if the languages are different!

It might not be easy to find these sites at first; however, if one translates brainstem glioma or pontine glioma (in a translator site such as google translator) some time new worlds open up.  This is exactly what I did in French.   Putting "Tumeur du Tronc Cérébral" in the search engine was immediately rewarded by finding another DIPG site from halfway around the world.  Fortunately (for me) my commuter asked me immediately if I wanted to read in English.

 http://www.tumeurtronccerebral.com/

Funny, smart, sensitive Eva inspired her parents to make this site.  This little girl subsequent to developing a squint was diagnosed on June 5, 2010.  After a 7 1/2 month battle she died on January 20,2010.  

This site contains quite a bit of information- from understanding high and low grade brain stem tumors to different therapy options.   As with many parents starting sites, there are items trying to make a difference.   One such thing is a petition calling on more research for pediatric cancer research in France.  Another is a survey of parents who have had children with DIPG.   

Perhaps one of the most sought after items is a page about long term survivors.

http://www.tumeurtronccerebral.com/longs-survivants__15_.html

Most of these children are atypical in some way.  There are three points to that:

1) Prolonged survival does not mean a cure- just that so far these children have beaten the statistical odds.   Most of these children's site that have information about the tumor show that it is still there.

2) Not all families that came into the DIPG world are dealing with typical DIPGs so these rare but hope-giving kids are particularly important to those dealing with atypical DIPG.   

3) At least one child listed there is known to be typical by current standards- Dasia.

DIPG happens all around the world.   The world wide web is making all the difference in our being able to communicate and understand what is going on internationally.

Note- to understand the difference between typical and atypical DIPGs

http://dipg.blogspot.com/2013/03/typical-versus-atypical-what-does-that.html

New Trial- MGMT Cancer Gene Therapy Trial

One of the huge problems with chemotherapy is that it beats up normal cells- especially bone marrow.   The toxicity limits how far the doctors can increase dosages before it is just too much for the body.  One novel idea is to try to make the normal bone marrow cells more resistant to chemotherapy.  This is exactly what researchers are trying to do with a gene therapy which effects MGMT.  This will allow for increasing doses of temozolomide.

Dr Geoffrey McCowage at The Children's Hospital of Westmead in Sydney Australia has spent the last 15 years developing a trial which removes bone marrow stem cells, genetically modifies the NA repair protein and then reinserts them into the patient.   The hope is that this modification will allow a patient to withstand escalating chemotherapy while better targeting the tumor.   In fact, this is more than theory.    This first of kind therapy for brain tumor kids is open in trial.

This Phase 1 intervention is open to several different types of brain tumors including brainstem glioma of diffuse pontine type  and all the following recurrent tumors- medulloblastoma, ependymoma, atypical teratoid rhaboid, high and low grade glioma.

Erin is the first child enrolled.   You can read at-
 http://www.news.com.au/national-news/south-australia/brave-erins-inspirational-battle-against-cancer/story-fndo4dzn-122658419720
Last month she had reached a year from diagnosis- and in the picture she looks great.

The trial is being funded by the Kid's Cancer Project (formerly Oncology Children's Foundation),  Sporting Chance Cancer Foundation and the Australian governmental funding agency, The Department of Innovation, Industry Science and Research.

Erin is hoping to raise $50,000 for The Cure Starts Now for DIPG research.

Note- A similar trial seems to be open in the US at the NIH for adult glioblastoma patients.

References:

Brave Erin's Inspirational Battle Against Cancer-  http://www.news.com.au/national-news/south-australia/brave-erins-inspirational-battle-against-cancer/story-fndo4dzn-122658419720

MGMT Cancer Gene Therapy Project- http://www.thekidscancerproject.org.au/Research/Gene-Therapy-Trial.aspx

Cancer Gene Therapy Project (Westmead Research site)- 

http://www.chw.edu.au/research/groups/oncology/research_groups/cancer/

Australian New Zealand Clinical Trial Registry (ANZCTR)-

https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?ACTRN=12612000535875

NIH Adult Trial with Newly Diagnosed GBM- http://www.clinicaltrials.gov/ct2/show/NCT01269424

Running for a Brother

The words are all too familiar- "I never heard of DIPG before...."

And so starts Jackie's story of her brother.   Michael was diagnosed on October 22, 2012.   The sweet, kind, full of energy 12-year-old started with dizziness and instability one fall weekend.   The unimaginably swift decline happened in just one week- dizzy on Monday, wheelchair on Wednesday,  struggling to eat on Thursday,  hospice on Friday.  Monday only one week after diagnosis Michael died.

Jackie shares their personal, poignant story because today she is running a half-marathon in her brother's memory.   The Shamrock Half-marathon in Virginia Beach donates money to cancer causes, however Jackie wants to be specific.   She wants to destroy DIPG.   She chose to donate her money to a foundation started because of another 12-year-old taken by DIPG- Aimee's Army.  Aimee's Army sponsors events to create awareness and fights for research about early detections.

Jackie, thank you for raising awareness of DIPG.   Congratulations on raising 3x's your goal.    Good luck crossing the finish line.

References:
Destroy DIPG for Mike-  http://www.active.com/donate/destroyDIPGforMike

Aimee's Army- http://www.aimeesarmy.com