Rare Cancer Stories
Share |
Home   Support   Story   Forum Support   Info   News & Clinical Trials   Cancer Dictionary   Events Calendar   SiteMap   Twitter   Facebook

© Desmoplastic Infantile Ganglioglioma

Read Robin Britton's Desmoplastic Infantile Ganglioglioma Informational Website

Join Robin on our Childhood Brain Tumor Support Forums

Diagnosis: My daughter Elise was born in April 2001, two weeks late and nine pounds heavy. She was a fat and happy baby and we were grateful to add her to our family. At six months of age she had her well-baby checkup and everything was fine, except she was in the 90% range for both weight and head circumference. Two weeks later my husband took her back to the family doctor because her soft spot was bulging slightly. We weren’t too concerned, and thought better to be safe than sorry. The family doctor checked her out and measured her head again. Her head circumference had grown ¾ of an inch in two weeks. He told my husband that she may be having problems draining fluid (csf) and sent them on to our local Children's Hospital. In the Emergency Room they did a CAT scan to see if the ventricles which drain fluid off of the brain were blocked, and boy were they. A massive brain tumor took up almost the entire right side of her brain. In the next two hours Elise was admitted to the hospital and scheduled for an MRI. 

Desmoplastic Infantile Ganglioglioma
After the results of the MRI were back we met with her neurosurgeon, neurologist, radiologist and oncologist we decided to let them operate and try to remove the tumor. She was scheduled for surgery at 8:00 am the next morning. The operation took about 9 hours and removed about 80% of the tumor mass. Portions of the tumor on her brainstem and her optic nerve on her right eye were deemed inoperable. Slides were sent off to John Hopkins to confirm the pathology and Elise was diagnosed with Desmoplastic Infantile Ganglioglioma (DIG). I thank the powers that be everyday that we got the neurosurgeon we did. She could have ended up blind in her right eye and paralyzed from the surgery, but because our surgeon was conservative she can see and only has weakness (hemiplesia) in her left arm and leg.

After Elise was out of PICU we started reading journals about her type of cancer. My husband is working on his PhD in Biology so we had access to the university’s medical library as well. Journal articles on her rare type of cancer were sparse to say the least. Everything we read – which wasn’t much – said that surgery was the curative measure. In essence if you could cut it out it would not come back. This advice did us no good, we couldn’t get all of the tumor out. There was no proven chemotherapy regime suggested in the literature and she was too young with a tumor far too large for radiation. In reading the journal articles and case studies we found that the tumor has essentially two paths it takes. Either the tumor grows slowly and benignly and can sometimes be treated with chemo, or it grows like gangbusters and the child is usually dead in six months to a year.

Treatment Elise eventually recovered from the first surgery. After that she had another surgery to have a VP Shunt placed in her head to adequately drain the fluid from her brain. About a month later she had yet another surgery to put a port-a-cath in her chest and make accessing her for chemo easier. By this time she looked like a pin cushion, so I was very happy to have the port in place. We started on the POG “Baby Brain Protocol” which is what chemo regime is given as a starting point when they don’t know what to do. The protocol was given in the hospital with Vincristine, Cisplatinum, Etoposide, and Cytoxin. She was miserable, and we were miserable with her. She did one full course of this protocol which is six weeks. By the time we had our first post-operation and chemo MRI her tumor had increased in size dramatically. It seems that the tumor had chosen the path of rapid growth, and they told us she probably had six months to live.

In this horrific situation, one of the best things that happened to us was getting Elise’s pediatric oncologist. Doctor Joann Sanders was honest, straight-forward, and answered all of my questions no matter how moronic or repetative. She told us that she had treated one other DIG child and that he had all of the tumor removed in surgery. She was smart enough to suggest that we needed to find another oncologist, a specialized pediatric-neuro-oncologist that had experience with partially resected DIG tumors. She explained to us that she would like to coordinate Elise’s care locally and would do anything needed of her to help. She asked around at the annual conferences, and phoned colleagues to help us find the right person. She has done, and continues to go way beyond the call of duty.

We decided to take Elise off of chemo for a few weeks and started the search for a specialized oncologist. We had her case reviewed by MD Anderson (which is no place for kids), St Judes in Tennessee, Texas Children's in Houston. After talking with Texas Children’s we decided to go on a new chemo regime of Cisplatinum in the hospital and Etoposide orally at home. We started the new chemo immediately and prepared ourselves for the worst.

Believe it or not we finally caught a break. In subsequent MRI scans the tumor slowed growth dramatically and eventually stopped growing all together. This was no magic concoction however. As a result of the chemo Elise was losing her hearing. In total we did chemo for a year before we decided to stop. It was my husbands questions mainly that made us stop chemo. We didn’t know if the chemo had killed the growth portion of the tumor and it was just in her head like a lump – in which case why continue the chemo and hurt the baby. The other option was that the tumor was still growing and that the chemo was just keeping it in check. Either way we wouldn’t know the answer until we took her off of the chemo, so we did. In the mean time we also kept up our search for a doctor with DIG experience.

It took us about a year and a half to find the right doctor in the US. We finally ended up at The Brain Tumor Center at Duke University in Raleigh North Carolina. Doctor Sri Gururangan is the pediatric neuro-oncologist that Elise now sees. His has another DIG child with a partially resected tumor who he is treating with chemotherapy. We are very happy to have found him. He directs Elise’s care, reviews all of her scans, and coordinates with doctor Sanders and us personally.

Now Elise has been off chemotherapy for six months now, all of her scans have shown no new growth in the tumor. In fact the tumor has continued to slightly decreased in size. Her quality of life has increased dramatically. There is no other way to say it – chemo sucks the life out of you. So our strategy now is to wait and see, and keep her off chemo and growing and laughing as long as possible. She is learning to walk, learning tons of new vocabulary, and socializing with other children again. Who knew that a two year-old with half a brain could know all of the words to Twinkle, Twinkle Little Star.

Desmoplastic Infantile Ganglioglioma
3-4-2005: I just wanted to update everyone on the good news from Elise’s last MRI.  The radiology report basically says that there is mild improvement in the tumor.  This type of tumor has three basic composition types 1. soft tumor tissue  2. fluid filled sacs called cysts  3. and hard nodular areas.  The two nodular areas are slightly improved, with a mild shrinking in the shortest axis and less enhancement overall (which is good).  The fluid filled sack behind her right eye also appears slightly smaller.  My favorite is the last statement on the report, “No areas of worsening were identified.  Otherwise stable examination.”  We are very happy to say the least.  Her next scan will be sometime in August-September.

I have attached Elise’s latest school picture.  She looks like such a little girl these days, and it is amazing how much hair she has.  We went to the open house at Jakob and Elise’s school last night.  Both of their teachers think they are doing very well.  We had lots of fun having the kids show David and I around the classroom.  I hope everything is going well with all of you and your families, and we are very thankful that we have good news to share.

From our personal experiences, I can tell you these things

You must become well educated on your type of cancer so you can speak the jargon of the doctors.
Doctors don’t always have the answers.
Your word, not the doctors, is how the final decisions must be made.
You are not alone, Stay Tough!

Read Our DIG Informational Website
Join Robin, Elise's Mom, in our Childhood Brain Tumor Forums