Brain / CNS Tumors Support Group

My 1 year old daughter, Vanessa has a tectal glioma. This was diagnosed when she was 9 months old after she developed hydrocephalus. After her initial MRI scan, the neurosurgery team felt confident that she was a good candidate for ETV (Endoscopic Third Ventriculostomy) which, if successful, would eliminate the need for a VP shunt. At first, the ETV seemed to be working, but suddenly, about 5 weeks after the procedure, her symptoms returned, and the hydrocephalus seemed to be back worse than ever. So they immediately performed surgery to place a VP shunt. That was a little over one month ago and she seems to be doing fairly well so far. Her repeat MRI was just last week and it showed that she has a subdural hygroma, which is a collection of CSF in the subdural space. It is not a huge concern right now, but it is something that they want to keep a close eye on. The hope is that as her brain re-expands to its normal shape, the fluid in the subdural space will just get re-absorbed. The other concern is that there seems to be a slight change in the tumor in this second MRI after only 3 months. This could be due to the fact that the structures in that area of the brain have shifted a little bit, but we are very worried. Tomorrow is our first official sit-down meeting with our neuro-oncologist (we met briefly in our hospital room after Vanessa's first surgery) and we have a lot of anxiety regarding what they might have to say. We are trying hard to remain positive and believe that the worst is behind us. I would love to hear how Emma is doing.

Since my last post I have great news to report. The MRI did turn out to be just better imaging of the tumor. She has been stable since diagnosis (2006). VanMark, I'm so sorry to hear of Vanessa's diagnosis -brain tumors are a scary beast. I am anxious to hear how you made out after meeting with the neuro-onc. I know your anxiety and absolute fear, and my heart and prayers go out to you and your family.
I am so fortunate and blessed to say that Emma is a typical 4 year old. She loves school, is active in soccer, music and gymnastics. She attends a french school so she is mastering english and french. From her early diagnosis of hydrocephalus, we learned a lot about this condition and have been active in making sure she met milestones in early development and are keeping an eye on academic and physical development as she grows. We joined the Spina Bifida and Hydrocephalus Association of Ontario (I believe you are also from Canada) and they have been such a wonderful source of information and support. If you haven't joined a similar association in your area, I highly recommend it. They have provided me with research literature, a binder of info to give to Emma's school to educate them on her hydro and what it might mean for her academic development and even a children's book about a boy with hydro who encounters a shunt blockage, requires some "fixing" and is then all better. I haven't yet introduced Emma to the book. She knows she has a shunt at the back of her head and a tube that goes into her tummy (I had her feel the catheter on her neck and chest) and just that she needed some fixing when she was a baby. I'll message you with more info as I could probably write a book! Can't wait to hear your good news about Vanessa -Sincerely, Amy

Thanks for your quick reply Amy! So we had our meeting with neuro-oncology yesterday and they are satisfied that there has been little, if any, change in the tumor since her first MRI three months ago. They are happy to wait 6 months to re-image it as long as she doesn't start having any neuro symptoms in the meanwhile. While the news is good, we were hoping (perhaps naively) that they would tell us that we wouldn't have to worry anymore and that everything looked great. I guess we are just having a hard time coming to terms with this as a life-long diagnosis that will need frequent and long-term follow-up. Vanessa is doing relatively well despite all of this - she has had some gross motor delay as a result of her increased ICP (and the weight of her head), but she is being followed by the Infant Development Team and is progressing nicely. You're right that we're also in Canada, we live in Vancouver and there are tons of resources here, we're just sifting through all of them right now trying to determine what would help us. I am also well-connected with resources in the community as I am a Registered Nurse at BC Children's Hospital. The one problem with this is that everyone just assumes that, because of my job, I automatically know everything (or at least more than the average family) about Vanessa's condition. I've had to ask more than one specialist to just talk to us like they would any other family. I'm very glad to hear that Emma is doing so well, and I think it's wonderful that you were able to give her a simple explanation to help her understand what her shunt is. Thanks again for your quick reply and I'd love to keep in touch as we continue on this journey.

Mark

Hi Mark,
I'm so happy to hear your good news of a stable scan! I must admit, I had crafted a reply to this post last week and after putting in a great deal of time, I lost my connection and my post and hadn't had a chance until now to try again.

The schedule doctors adhere to regarding MRI's on these type of tumors vary so much. In the many people I've met, some have had doctors order scans every three months for the first year and then every six months and then every 12. Our neuro had us repeat every 12 months right from the start.

I can relate very much with the difficulty in coming to terms with this diagnosis. Knowing that we will never be "out of the woods" is a hard reality to face. As well, I had great difficulty in accepting the fact that my daughter has a brain tumor...to me, "accepting it" meant that I was somehow okay with it, and I wasn't, for a long time. That's the best way I can verbalize it. I can't say I'm okay with the tumor, but I do accept the reality and I'm doing what I can as we venture on our path, wherever it may take us. I started finding some peace when I thought more on the notion that perhaps she was meant to come to earth with this tumor and we were chosen to be her parents because He knew that we would be the best to care for her and love her (I don't mean to offend or be preachy here if you don't share the same beliefs). In Vanessa's case, she couldn't be in better hands!

That is fantastic that Vanessa has a team to assist her in her early development. As Emma's hydrocephalus was detected at 6 weeks, we had a physical, occupational and speech therapist follow her throughout her first year of life. She met all her milestones so we were discharged. I'm sure Vanessa will amaze you how quickly she'll catch up on her gross motor skills-these munchkins are so resiliant!

How amazing that you work in a children's hospital and are so connected to great resources. The greatest stumbling block myself and others with this tumor have found is the lack of research and information out there. After meeting several people online, we shared a desire to compare notes, research and information and as you know tumor-speaking, it is apples to oranges unless you are comparing your specific tumor with someone else who has the same specific tumor. As a result, I had created an online support group through yahoo health, specific only to those with, or a have a loved one with, a tectal glioma. http://health.groups.yahoo.com/gro... It would be great to have you join as well. We have a good collection of links and files of information, and there has been excellent information in our posts about the experiences & knowledge we've shared in comparing symptoms, challenges, etc.

I'm glad to have met you and would love to keep in touch as well as we journey forward.

My best to you and your family, Amy

best wished to you and your families

Hi,

I am a 39 yr. old mom with what they think is a tectal glioma. I am going to go to Johns Hopkins Neurosurgery Dept shortly to get confirmation. The doctors have told me that they don't think it can be biopsied so they want to watch it. I am leary to do that without knowing what I am dealing with. I am having some neurological issues (which led to finding the lesion) such as vision problems, hearing issues, ataxia, balance problems, nystagmus, and headache. My lesion right now is only 5mm (they think) so if they can biopsy it without doing much damage I think I will let them. I would love any advice or suggestions that any of you have. This has been a very rough 2 weeks.

All the Best,
Heather

Hi Heather,

I am so sorry to hear about your scary news. As you have read, my 15 month-old daughter has a tectal glioma. I am sure that you are feeling a bit frustrated at the lack of information out there as we were when trying to research this condition. It is a particularly rare type of tumor, but from everything I've learned it is also quite treatable. In my daughter's case, as is most common with a tectal glioma, the tumor blocked a structure known as the aqueduct of sylvius that normally allows cerebralspinal fluid to drain out of the 3rd ventricle at the centre of the brain. This led to a condition called hydrocephalus, which is basically a collection of fluid that puts brain structures under pressure and can cause all kinds of symptoms like nausea, dizziness, unsteadiness, headaches, and all kinds of scary things. In my daughter's case, they first attempted a procedure called a 3rd ventriculostomy, which is intended to create a new pathway for the CSF to drain. Unfortunately, this procedure was not successful and a few weeks later they implanted a device called a VP shunt, which is basically a tube that is inserted through a very small hole in the skull into the 3rd ventricle then tunneled under the skin to her abdomen. This tube allows the extra CSF to drain away from the brain to the peritoneal cavity in her abdomen and has, so far for us, been very successful. When we asked our doctors about the long-term management of my daughter's condition, they were all quite optimistic that she may not require any further interventions, aside from a possible revision of her shunt (which is very commonly required at some point). The plan is to monitor her with MRI scans every 6 months for the next year or so and then annually if there is no sign of change in the tumor. The other thing that they told us regarding her tumor is that these types of tumors, if they do start growing, are easily managed with a non-aggressive form of chemotherapy (I think the drug that they mentioned specifically was vinblastine) that can be administered on an outpatient basis, and has very few of the side effects that are commonly associated with chemotherapy. They also said that in very rare cases where the tumor is growing and chemotherapy is not working, radiation therapy is an option. One thing that I have learned is that with this type of tumor, because it is on the brain stem which is an extremely delicate and important part of the brain, surgical removal is almost never an option as the risk of damaging vital structures is just too great. In my research it seems that most neurosurgeons aren't even comfortable taking biopsies of these tumors for the same reason. I hope this is not too much information for you to digest all at once, and please remember that management in adults may be different than in children, so some of the information above may not apply for you. Please let us know how you are doing and please know that we are thinking of you and are including you in our prayers. Take care of yourself.

Mark