10 and 15 year Survivors

Meet Connor and Tori.

Their names have been around the internet for some time.  I absolutely believe that their parents were told the awful prognosis of DIPG at diagnosis and although I have never seen the MRIs I don't doubt that the lesion in these children's brains were diffuse and intrinsic and pontine.

Connor was just 5 weeks old when he was diagnosed with a DIBSG (diffuse intrinsic branstem glioma).  The parents knew something wasn't right about a week earlier when he wasn't latching on and seemed exhausted.   A seizure sent him to Cincinnati Children's ER.   During the admission for failure to thrive a golfball size tumor was found in his brainstem.   At that young age there were no therapeutic options.  He was sent home with a feeding tube to die.  In those first  two years he had spectroscopy scans which showed high levels of choline and other cancerous indications but it didn't change.   As far as I can tell his last MRI still had residual tumor at age 2.  In September, this happy healthy boy will have his 10 year anniversary from diagnosis.    His mom has a caringbridge site to give hope to other DIPG parents.

Tori seems like a regular teenage girl.   However, not long after she was born that thought that we would ever know seemed beyond impossible.  Tori was born in June 1998 and a few weeks later was found to have some typical DIPG symptoms.   Her eyes had trouble tracking and the side of her face seemed stiff.  AN MRI showed a brainstem glioma (that is what they all were called at that time).  The doctors told her parents she would die.   With no conventional options, they searched for alternatives.  This year she wrote her own update.  This fall will be 15 years since diagnosis for her!

One can ask a million times but no one knows why these children have survived.  What we do know is that there have been other cases of spontaneous regression of diffuse pontine lesions in neonates.   It has been reported in the literature before.

A case from Germany is remarkable similar to the two above.  A 7 week old infant was found to have an MRI "strongly suggestive of diffuse pontine glioma".  By 27 months the lesion was gone.

A slightly different situation (also from Germany) is the first reported case of spontaneous remission that I am aware of.   A 2-year-old boy was found to have a large, diffuse pontine tumor extending into the midbrain.   A biopsy showed a fibrillary astrocytoma.   He received a shunt but no other conventional therapy.   By the age of 6.6 years the tumor was no longer visible on imaging.

In 2005, two US neurosurgeons reported two cases of spontaneous regression of diffuse brainstem lesions in neonates.  These two newborns were found to have limb weakness and cranial nerve abnormalies.  MRIs in the first week after birth revealed a large pontomedullary tumor.  Neither child received conventional treatment.   The authors related that the children were normal at age 4 and 10.

A followup letter to the editor to the above case report came from Texas Children's relating a similar story although the child presented at 11 weeks of age.   At the time of publication she was 12.

These authors stressed that nearly all patients with diffuse brainstem lesions do poorly-  including neonates.   In 2008 Sick Kids published a case report on  two infants with "large, expansive, non-enhancing, intrinsic pontine masses diagnosed as DIPGs.   They were treated only with supportive care and died by 2 weeks of age.   One of the children underwent autopsy and was found to have a brainstem PNET.

We have no idea how often this happens.  This is why the DIPG Registry  is critical in understanding this disease.  Both families and physicians can enroll patients including children who have died.

The bottom line is:  1) we really don't understand DIPGs, and 2) there are children who have been diagnosed with DIPGs-based on the incomplete knowledge that we currently have- who truly have had spontaneous regression of their tumors and 3) most of the children with report spontaneous regression have been very young.

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.