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.
