Treatment of optic nerve tumors

Once again, I am on a mission to find the latest information in dealing with optic nerve tumors.

My 16 year old daughter, Chloe, has three eye tumors. One on the optic nerve, the other two in the other eye are near the fovea.

She had one laser surgery last fall. Her vision was affected, so we have been reluctant to proceed. We have been in a watch and wait mode. However, she was recently seen by our retina specialist and it appears that they have grown. He feels like the tumor will grow and get to a point where laser surgery will not be an option anymore. It will be too big and "juicy."

Clincial drug trials may be an option for us. We are looking into this now. I feel like the clock is ticking and that we must make a decision soon.

I would very much appreciate your feedback regarding drug treatments and/or laser surgery.

I think I probably posted this exact message a year ago, but I know a lot has changed since then.

Thank you in advance for sharing your experiences.

God Bless,

Alison

By MaryP
Posted July 14, 2008 at 11:19 pm

Thank you for the discussion. My daughter also has an optic nerve tumor. We also tried laser and she ended up with scar tissue that caused a deterioration in her vision. She ended up having a vitrectomy which helped her vision, but did nothing to get rid of the tumor. We are in your same situation and would love to also have a good answer.

By joyceg98
Posted July 15, 2008 at 12:00 pm

I asked Dr. Emily Chew of the U.S. National Eye Institute to write an article about this topic, which will be published in the September newsletter.

I have put the draft on the internet for you and others to review, and let me know if you have additional questions not covered by this article that we should get answers for before we publish it.

Please see
http://vhl.org/newsletter/vhl2008/08cx_chew.php

I will appreciate your comments and questions.

Dr. Singh is contributing a companion article on photo-dynamic therapy which has not yet been received. If you wish, I can put it out for early review as well. I am expecting it about August 1.

Best wishes,
Joyce

By joyceg98
Posted July 15, 2008 at 12:16 pm

Alison, our experience with laser surgery is that the tumor absorbed the laser energy and swelled, pressing on the optic nerve and causing some permanent damage to the nerve. So approach laser with enormous caution.

My son has recently had some photo-dynamic therapy (PDT) with Dr. Jay Duker at Tufts in Boston. The PDT does not cause swelling. His tumor is far advanced (identified 20 years ago and has caused vision issues for the last 10 years) so the treatment has not improved his vision, and it's early to say how much fluid it may have reduced (he's off the chart on the OCT that measures the fluid accumulation), but that seems to be the best method we have tried to date.

Dr. Arun Singh in Cleveland also believes that PDT is the best existing option. Whatever you try, approach it very cautiously and with very small doses. There is always a concern that it might damage the nerve, so it would be best to speak with someone who has experience with VHL (like Duker or Singh).

Dr. Duker also tried some focused radiation therapy with my son's eye, which might be helpful for Chloe.

http://www.tuftsmedicalcenter.org/OurServices/Ophthalmology/default

http://my.clevelandclinic.org/staff_directory/6/staff_5025.aspx

Best wishes,
Joyce

By AEckerman
Posted July 15, 2008 at 8:01 pm

Thank you, Joyce, for this helpful information.

I would very much like to see Dr. Singh's article if you don't mind. You can send it to me privately if your don't want to post it yet.

Regarding Dr. Chew's article, I am just wondering if it would be a good idea to include Sutent's possible side affects? I know I am curious.

Thanks~
Alison

By marie3
Posted July 15, 2008 at 9:49 pm

Hello Alison,

How are you? I was in Dr. Chew's Lucentis trial. I received 14 intraocular injections. My tumor continued to grow. My retinal specialist sent me to Boston for proton beam radiation. I received 5 treatments. Approximately a year later, I have neovascular glaucoma due to the radiation. This past January I almost had my eye removed due to the severe pain. Right now, I do not have pain, although the pressure is at 55. I also do not have vision in that eye. My vision was gradually deteriorating prior to radiation. A year after radiation, my eye started to hemorrhage and I lost all vision. Right now I'm playing the waiting game.

Take care,
Marie

By ReneeMichelle
Posted July 16, 2008 at 11:20 am

Hi Alison (and all)
I also have tumours on both optic nerves as well as one in my right eye near the fovea. The one near the fovea has caused all of the trouble. I had laser surgery on it many times over several years and the scar tissue that has built up is now pulling on my retina. Dr Chew felt the laser was no longer effective and so we tried Avastin injections. I had three injections and the tumour just got worse until the fluid leakage spread right across the fovea and erased my central vision. Then we tried PDT (Photo Dynamic Therapy). My vision improved from 20/800 to 20/80. I've had PDT three times and I don't believe there have been any side effects.

I have not had any treatment to my optic nerve tumours, however. They are growing, but VERY slowly, and I don't think they are affecting my vision as of yet.

Good luck with Chloe. You are always in my prayers.

Love, Renee

By kez02m
Posted July 16, 2008 at 7:44 pm

hi Iam interested as well my step daughter also has a tumor on her optic nerve and they are just watching it they cant hit it with laser as there is a risk in losing her sight which it worrys her as her father has lost his sight kerry

By AEckerman
Posted July 16, 2008 at 8:00 pm

It sounds like PDT may be something to seriously consider. Is this therapy available only at certain hospitals? Does anyone else have any direct experience with this modality? Good or bad?

Thanks so much,
Alison

By AEckerman
Posted July 16, 2008 at 8:05 pm

Good to hear from you, Renee.

Who did the PDT? It is wonderful to hear that you haven't experienced any negative effects - I like that part.

Keep in touch,
Alison

By ReneeMichelle
Posted July 17, 2008 at 2:08 pm

Actually, I had PDT here at home. My own local opthalmologist did it for me, Dr. Leland Dhurjon. He's very good. We're lucky to have him way up here in Northwestern Ontario. The drug required for the PDT is not covered by Ontario Health Insurance Plan (OHIP) and it costs $3000 per treatment, but Dr. Dhurjon contacted the drug company and they donated it to me! Thank goodness. Saved me $9000 all ready.

I think most retinal specialists probably do PDT. It is more commonly used for Macular Degeneration, and so isn't a rare treatment. From what I understand.

I should mention, right after the PDT my vision got slightly worse for about three weeks before it started to improve. And I think it was 5-6 weeks before it stopped improving. It didn't make my vision perfect, but it made it better. I am very glad I tried it. Although, the tumour I had treated was not one of the ones on my optic nerves. I hope someone with that specific treatment can comment. Anyone??

By marie3
Posted July 18, 2008 at 10:52 pm

Hi all! I forgot to mention in my earlier post ----my tumor is on the optic nerve. Before I had proton radiation, I consulted with Dr. Chew. She knew the Doc in Boston who performed the radiation. All the doctors I consulted with said radiation was my last resort. Lucentis injections (cousin drug to Avastin) didn't work.

Marie

By AEckerman
Posted July 19, 2008 at 10:36 am

Hey Marie,

Do you mind telling who the doctor was in Boston that Dr. Chew referred you to?

Did you ever try the PDT before the proton radiation? Was that ever suggested?

Thanks!
Alison

By marie3
Posted July 21, 2008 at 11:59 am

Alison,

The doc at Boston Mass. General is Evangelous Gragoudas. He came highly recommended from my Retinal Specialist, as well as Dr. Chew. I always consult with Dr. Chew via E-mail before I do any thing to my eye. PDT was mentioned to me in the past, but not as a real option. Only two hospitals in the country perform Proton Beam Radiation in the country. Boston Mass. General and Lo Lalinda (spelled wrong) in California. Proton radiation was my last hope to save the eye. It was explained to me like this...... Traditional radiation has a point of entrance and exit, therefore damaging healthy tissue. Proton radiation has a point of entrance and stops at its target--no point of exit. Also, it is suppposed to be more accurate and localized when reaching its target. The treatment is the same for ocular melanoma---- EXCEPT with ocular melanoma you receive a higher dose of radiation PLUS prior to radiation, discs are sewn into the eye, surrounding the tumor. In my situation, 1 to 1 1/2 years later, the tumor is shrinking BUT the eye is internally hemorrhaging. Even in a dialated exam the docs cannot even see through to the back of the eye due to all the blood that is present. We're not 100% sure what exactly is bleeding. Tumor? Damaged tissue?
I'm not trying to detour anyone from radiation. In my situation, I was told by NIH and by my very reputable doc, "If you do nothing you will lose your eye. Radiation is the last hope in saving the eye. You may lose the eye even after radiation." Right now, the eye is blind , but it is not painful and it cosmetically looks good. I take it one day at a time, knowing that surgery could be near. I feel very fortunate to have one good eye. I know many people are facing issues
with both eyes. Sometimes having VHL makes me feel like LIFE STINKS (I have better words), but then sometimes it makes me appreciate the small things in life.

Sorry to babble so much. Hope this helps.

Be strong!
Marie

By AEckerman
Posted August 14, 2008 at 8:00 pm

Sutent has been mentioned as a possible treatment - usually reserved for advanced RCC. I would love to hear from anyone who has tried this drug.

We are going to see Dr. Chew in one month, so trying to learn about every possible treatment.

Thank you all so much for your help!

Alison

By donnab
Posted August 15, 2008 at 8:30 pm

Good Luck Allison and I hope that you are able to find a treatment that will at least do no more damage to your daughters vision.

I was also in the Lucentis trial with Dr. Chew in 2005. I have an optic nerve tumor in one of my eyes. After receiving treatments for a year my vision deteriorated to the point that I had to be withdrawn from the Study. I received radiation treatment in June/July of 2006 and seems to have stopped/slowed tumor activity for now. I don't think I have lost any vision, but I have not gained any vision either. I had direct beam radiation for 2 weeks, every day. The only side effects I had was an area of hair falling out on the back side of my head where the radiation "came out" of my head. But, they had no idea what all might happen because it can vary from person to person.
At my last visit with my Dr. he mentioned possible PTD therapy if my tumor does not stop the leaking. He also said that PTD has improved since we first considered it back in 2004. Sounds like that may be a viable option to consider after hearing a few of the stories.

Know that your family is always in my prayers and I will especially pray for a good solution to your daughters treatment and vision.

By marie3
Posted August 15, 2008 at 8:46 pm

Alison,

I just remembered this....at one point Dr. Chew mentioned the possibility of intraveneous Avastin injections versus intraocular injections. Although there are side effects of high blood pressure. Back then, she felt that Avastin, injected systemically, would reach the blood vessels better and remain in the eye longer.

I never had this done. I don't know what's going on with it to date.

Donna B., did you have this done?

Marie

By marie3
Posted August 15, 2008 at 8:54 pm

Donna B.

It's funny. As I was typing my message to Alison-you posted yours to her. Your message wasn't there 10-15 minutes ago. We must be sending subliminal messages to each other.

Marie

By AEckerman
Posted August 15, 2008 at 10:31 pm

Thank you so much, Donna, Marie, Joyce and Renee, for helping me muddle through this. You all are such wonderful friends both on and off of this site! =)

Donna - you had direct beam radiation that is different than proton radiation, correct? It sounds to me like proton radiation would be safer. I am only saying this because of the "exit" issue that Marie mentioned.

Joyce - The focused radiation you mentioned, is that the same as direct beam?

I have also heard of "Strontium" (sp?) radiation, which is done in the OR through an incision. Anybody familiar with that?

I have talked to one person who had success with the systemic IV Avastin. She didn't experience any side effects. Is there anyone else who has done this and would like to share their experience?

One of our local retina specialists recommended Avastin and PDT. Our other local retina specialist said PDT was not an option in her case and prefers traditional laser - he did not explain why. They both feel like we can no longer wait, as there is lipid trailing to the macula and it is just a matter of time before her vision is affected. Her vision went from 20/20 to 20/400 after the laser last fall, after several months it improved to 20/40 which is where it has remained. Of couse, the 20/40 is with her head moving around to achieve that.

With 60% of all VHL tumors being in the eye, I wish that we had direct access to a data base that we all could see clearly what has worked and what hasn't. I could continue this discussion privately, but I know there are others that might be benefitting from this much needed information as much as I am. So I am attempting to keep this discussion alive.

God bless,
Alison

By minimammax4
Posted August 16, 2008 at 3:50 pm

Hi Alison,
You and I have spoken about the systemic IV Avastin injections. I whole heartedly agree with giving that a try, if it is possible. With no side effects, and the tumors basically remaining "stable", it's the best option we have came across so far. Sutent was not good for our daughter with all the side effects she experienced with it. We have been doing the Avastin this way for almost 3 years now. It's all thumbs up for us! I hope you are able to receive more feedback on this form of treatment.

By joyceg98
Posted August 17, 2008 at 11:21 pm

Alison, it's not just a matter of assembling data and checking a database to see what works. You are dealing with a very specific, quite unusual problem. A minority of people with VHL are dealing with tumors on or near the optic nerve, and especially ones that are exudating.

You have done a great job gathering perspective. Now it's time to choose a specialist and work with that specialist to choose a path. The first choice may or may not work for Chloe, in which case you and your chosen specialist together will choose option #2.

Best wishes,
Joyce

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