DIPG/DIPT Discussion

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A searchable blog on DIPG research, DIPG news, recent publications, DIPG Foundations, DIPG researchers, clinical trials as well as other issues relating to Diffuse Intrinsic Pontine Tumors- both Diffuse Intrinsic Pontine Gliomas (DIPGs) and Atypical Pontine Lesions (APLs).

For parents, family and friends of children with DIPG looking for information and connection to others dealing with DIPG please check the buttons on the right hand side for resources.

Monday, April 1, 2013

Are there survivors?

Are there any survivors of DIPG- even just one?

This is the fervent plea of so many parents whose children were just diagnosed with this terrible tumor. A  search of the internet yields conflicting information.  Here are some statements I have found:
*There are no long term survivors of DIPG.
*There are long term survivors.
*The long term survivors are atypical.
*If there are long term survivors then they were mis-diagnosed.

What is a parent to think?  Who do you believe?   Why are these statements so divergent?  Is there an answer one can trust on survivors?

My short answer:
*There are long term survivors of DIPG (meaning more than 5 years).

*Most of the reported long term survivors are atypical in some way especially the very young (under 3), prolonged symptoms before diagnosis or atypical features on imaging- but not all.

* It is hard to say that a long term survivor was misdiagnosed since the diagnosis is based on imaging. The assumption has been if the lesion is uniformly diffuse and encompasses a large part of the pons then the lesion is a glioma.  It is then called a DIPG.   Since we have not have routine biopsied it is impossible to know the histology so all might not be gliomas.   However, one can tell on imaging if the lesion is diffuse, intrinsic and pontine.   It might be semantics but it seems hard to be a misdiagnosis per se but rather currently there is an inadequate understanding of these lesion.

I am virtually certain that the parents of prolonged survivors were given the exact dismal prognosis as those parents whose children had only a short time.   I think it is extremely unfair to then go back and tell these parents that "well, it wasn't really a DIPG".   It seems more valid to say that we don't understand these tumor.  Some unpredictably and inexplicably do better than the vast majority of others.

So, why can I be so certain that there is at least one term survivor?   One doesn't need to take my word for it.   I would recommend viewing a 2009 video of a top St Jude researcher, Alberto Bronsicer.
http://justonemoreday.org/DIPGConference/NewClinicalTrialsfromStJude.html
(go in to 15:49 minutes)

Transcript:
I swear to you- typical brainstem glioma.  I work her up- was one of my first patients at St. Jude.  Full blown findings.  We have spectroscopy.  You name it.  This is the MRI to show and 5 years later.  She has some sequela of her treatment.  One thing I point to all my parents- she never had complete response.  A partial response.   You see changes there 5 years later and she is out and about.

So yes, there is at least one long term survivor of what appeared to be a very typical DIPG.  Unfortunately, the vast majority of children with these tumors will follow this path but for those that find hope in just one- here is one.

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