Esthesioneuroblastoma

SIRENAF42 said:

Esthesionueroblastoma
I was diagnosed with Ethesio... in Jun 2008, underwent suregery to remove the tumor and then 6 weeks of radiation at MD Anderson in Houston TX. My last treatment was Oct 1 2008. My 30 day check up was in NOv and my drs were very pleased with the results. I go back in March for my next check up... I am sooo excited that I found someone else with my type cancer.. I cant wait to communicate with you.
Sirena

Hi Sirena,  I was just diagnosed a couple weeks ago.  I had been having some conjestion for several months and had gone through multiple rounds of antibiotics.  My family physician referred me to an ENT.  My CT scan showed complete blockage on my left side.  I had an MRI the next week.  Last Friday I had the majority of the tumor that was in my sinuses taken out.  This Friday I am going back to surgery to have the remainder of the tumor taken out.  They have to remove a small bone to get to it and my doctor tells me I will lose my sense of smell.  Fortunately they are going to be able to do the procedure endoscopically.  I will get a lumbar drain Friday morning then have surgery in the afternoon.  This whole process has been very fast and extremely frightening.  I am otherwise a very healthy 52 yr old with 4 kids.  All I have been able to think about is will I be able to see my children marry and hold my grandkids someday.  It's reassuring for me to hear about survivors of this terrible disease.  Any insight you or anyone can provide will be greatly appreciated. 

Mona4 said:

New Diagnosis

Hi Sirena,  I was just diagnosed a couple weeks ago.  I had been having some conjestion for several months and had gone through multiple rounds of antibiotics.  My family physician referred me to an ENT.  My CT scan showed complete blockage on my left side.  I had an MRI the next week.  Last Friday I had the majority of the tumor that was in my sinuses taken out.  This Friday I am going back to surgery to have the remainder of the tumor taken out.  They have to remove a small bone to get to it and my doctor tells me I will lose my sense of smell.  Fortunately they are going to be able to do the procedure endoscopically.  I will get a lumbar drain Friday morning then have surgery in the afternoon.  This whole process has been very fast and extremely frightening.  I am otherwise a very healthy 52 yr old with 4 kids.  All I have been able to think about is will I be able to see my children marry and hold my grandkids someday.  It's reassuring for me to hear about survivors of this terrible disease.  Any insight you or anyone can provide will be greatly appreciated. 

Hi Mona,

My husband's battle with esthesio began in 2008. After two surgeries, radiation, and chemo, by all appearances, it appeared as though he was in remission. Then in 2013, the neurosurgeon picked up on some changes in his surveillance MRI. Then he had an abnormal PET that showed a glow in one of his lymph nodes and an area in his sinus that had been negative before. Two surgeries later; we had good news and bad news. The good news was the sinus appeared to be negative; the bad news was two lymph nodes were positive. As we were not esthesio newbies; we emailed all the experts and got their opinion via email and saw one of them at UVA. My husband Tom decided this time to go radiation only which he seemed to fair much better this time around. Tom just had a PET yesterday and we will be seing his radiation oncologist tommorow at Hopkins. 

I have a few lessons learned that I can pass on to you.

1. This type of surgery is very complex and requires typically a neurosurgeon and ENT (sinus specialist). That's a red flag if there is not two. 

2. If the surgeons are doing it right, there should not be a need for a second surgery. The surgeon should be sending off frozen sections while you are on the table until it comes back negative. That's what they mean by a negative margin. We learned that lesson the hard way. The fact that our first surgeon was doing it solo should have clued us. The second one was 19 hours until they got negative margins.

3. This type of surgery should be done at a big teaching hospital not some little community hospital that is not up to snuff with current evidenced based medicine. There are several experts in the field and those are the folks that are publishing. UVA has Levine, Hopkins has Gallia, there was a few other good ones- (a) University of Pitt, (b) MD Anderson in Texas. 

4. Even if you don't have cancer in your lymph nodes, they should radiate the neck prophylatically. Apparently that's been pretty standard for a while. Learned that lesson the hard way too.

5. Have your surgeries and your treatments (i.e. chemo, RAD) at the same facility-another mistake we made.  Things get missed more when you have doctors at two different facilities . This practice leads to delays or poor communication hand-offs.

6. Do your research. Even if you feel like you're reading German..Google terms you don't understand. Here is one of the articles that I looked at last time. http://www.ajnr.org/content/32/5/929.full.

7. Follow-up surveillance is key. PET's and MRI's are the new norm for you. 

8. Ask your doctors questions if something does not sound right or bring a family member who will. You have to be your own advocate. 

9. I'm a nurse and I believe in this one the most. Always have a family member stay with you in the hospital. They can keep a closer eye on you and act as your advocate. 

10. Don't be afraid to ask for help from your loved ones. People have a tendency to help you out when it's convenient for them. Sometimes though that's not when the treatment is or the doctor is available. Tell people exactly what you want from them.

In regards to Sirena; I don't know if she is still active with this website. If you look at her postings, it appears her last one was December of 2011. Not sure what that means.. 

Lastly; I hope you're doing okay Mona. I would imagine if your doing chemo and/or RAD, you should be finishing up soon. If you're going with both, be prepared to be tired for a year. No kidding on that one. Keep your chin up and fight the good fight. You can do it!

Best,

Karla Platt

mom2.4 said:

This is my first
This is my first introduction to this web site. I'm glad to have found it, especially since ENB is so rare. Each of the posts have been very encouraging.

My daughter was diagnosed in Oct. of 2007 with ENB (Kadish stage C), at the age of 13. She had tumor (4cm x 5cm) resection at Seattle Children's. She had clean margins except where the tumor had invaded the fatty tissue around the eye orbit. She then went for proton beam radiation at MD Anderson Proton Center (for 8 weeks). Two months after radiation ended she had her follow-up appt. and both the MRI and biopsy showed irregular cells, which the doctor's decided was either residual or recurrent ENB. She then underwent 4 rounds of chemo at Seattle Children's. Three different types of Chemo were used: carboplatin, ifosfamide, and etoposide. She has been cancer free since July 2008. We have had amazing doctor's through the Seattle Cancer Care Alliance. Her surgeons are both from Harborview in Seattle, and have done an amazing job at tumor removal and reconstruction.

For those of you who had resection followed by radiation, what stage was your cancer and did the surgeons get clean margins?

My daughter continues to have PT scans and MRI's every 3 months. Although at her last appt. the Oncologist said that she could move to every 4 months and do MRI's only, since she has been cancer free for nearly 2 years.

I would highly recommend Proton Beam radiation for anyone with ENB. It has very little splatter and a very low exit dose, therefore has fewer side effects than traditional photon radiation. My daughter did not lose any hair, nor sense of smell, and did not have negative salivary side effects.

I hope this is helpful. Everyone's comments have been helpful and encouraging to me. If I can be a help to anyone going through what we went through, I'd be more than happy to.

Hi My husband has just been diagnosed and have Dr Kris Moe and Ferrarria at UW medical. Were they you daughters surgeons. They mentioned to us a young patient?

Thanks for any anwsers you might have.

Best

Melissa

KP said:

Get Ready to Fight The Big One Mona

Hi Mona,

My husband's battle with esthesio began in 2008. After two surgeries, radiation, and chemo, by all appearances, it appeared as though he was in remission. Then in 2013, the neurosurgeon picked up on some changes in his surveillance MRI. Then he had an abnormal PET that showed a glow in one of his lymph nodes and an area in his sinus that had been negative before. Two surgeries later; we had good news and bad news. The good news was the sinus appeared to be negative; the bad news was two lymph nodes were positive. As we were not esthesio newbies; we emailed all the experts and got their opinion via email and saw one of them at UVA. My husband Tom decided this time to go radiation only which he seemed to fair much better this time around. Tom just had a PET yesterday and we will be seing his radiation oncologist tommorow at Hopkins. 

I have a few lessons learned that I can pass on to you.

1. This type of surgery is very complex and requires typically a neurosurgeon and ENT (sinus specialist). That's a red flag if there is not two. 

2. If the surgeons are doing it right, there should not be a need for a second surgery. The surgeon should be sending off frozen sections while you are on the table until it comes back negative. That's what they mean by a negative margin. We learned that lesson the hard way. The fact that our first surgeon was doing it solo should have clued us. The second one was 19 hours until they got negative margins.

3. This type of surgery should be done at a big teaching hospital not some little community hospital that is not up to snuff with current evidenced based medicine. There are several experts in the field and those are the folks that are publishing. UVA has Levine, Hopkins has Gallia, there was a few other good ones- (a) University of Pitt, (b) MD Anderson in Texas. 

4. Even if you don't have cancer in your lymph nodes, they should radiate the neck prophylatically. Apparently that's been pretty standard for a while. Learned that lesson the hard way too.

5. Have your surgeries and your treatments (i.e. chemo, RAD) at the same facility-another mistake we made.  Things get missed more when you have doctors at two different facilities . This practice leads to delays or poor communication hand-offs.

6. Do your research. Even if you feel like you're reading German..Google terms you don't understand. Here is one of the articles that I looked at last time. http://www.ajnr.org/content/32/5/929.full.

7. Follow-up surveillance is key. PET's and MRI's are the new norm for you. 

8. Ask your doctors questions if something does not sound right or bring a family member who will. You have to be your own advocate. 

9. I'm a nurse and I believe in this one the most. Always have a family member stay with you in the hospital. They can keep a closer eye on you and act as your advocate. 

10. Don't be afraid to ask for help from your loved ones. People have a tendency to help you out when it's convenient for them. Sometimes though that's not when the treatment is or the doctor is available. Tell people exactly what you want from them.

In regards to Sirena; I don't know if she is still active with this website. If you look at her postings, it appears her last one was December of 2011. Not sure what that means.. 

Lastly; I hope you're doing okay Mona. I would imagine if your doing chemo and/or RAD, you should be finishing up soon. If you're going with both, be prepared to be tired for a year. No kidding on that one. Keep your chin up and fight the good fight. You can do it!

Best,

Karla Platt

Hello Karla, 

I just read your post here.  I am my oldest brother's advocate in this recent dx of ONB/ENB.  I work am not new to the world of cancer as our father had GBM and was dx in 2011 and passed in 2013.  To have my brother be dx with a tumor is just so frustrating and hard on us all.. especially him.  

We are in the process of choosing his surgeon/facility.  I live in Seattle, WA and he's in Oregon.  I work for BrainCancer.org and helped my dad a great deal with his brain tumor and work in teh field for last four years now.  I know a lot and can be of great help to my brother in this.  Since you have a lot of experience in this, I would love to talk with you more.  

We are considering UCSF and John Wayne Centers in CA.  I have reached out to Dana Farber...not much experience and would like to talk to MD Anderson and others... You mentioned several in your post... 

My brother's is a Grade 2 pathology and we're awaiting Kadish Staging.  It has minimal intradural extension and is mostly confined to endonasal cavity and involves the septum primarily.  He will need a periocranial flap.  Unsure on radiation and chemo... but likely will do radiation for sure and wanting to get opinions on chemo and what IS working to keep these at bay long term!  HE is an otherwise health 54 year old with great outlook since a negative pet scan.  

Sounds like the neck radiation is a strong consideration... Since your husband had radiation initially after surgery, what type of radiation did he have after recurrence in lymph nodes?  We were told they would likely max out on radiation in first go around... 

Would love to talk more.  You can email me at mmbarrett@me.com . 

Thank you and all the best to your husband. 

Maria Barrett

Hi This is the story about my Dad. He was diagnose with Esthesioneuroblastoma is 1992. It was stage C grade 3. It was extended to orbit, intracraneal compartiment and base skull. He went through hell. He had a major Surgery when they remove the tumor. He suffered complications after the surgery he had an infection with intracraneal hemorragea. He survived. 

He went through the maximun radiation that a person can received. He got almost a full 6 months of radiation. He lost his nose so they had to do facial reconstruction. However, he is still alive. Every year he goes back to be re-evaluated and no signs of the cancer back. Finally 4 years ago the doctor clear him. He had so many limitations that make his life a litle difficult at times. He has to use irrigations for life since he lost all the nasal cavities, his base skull was removed and he had a metalic for replacement and he gets so many headaches due to all the damage and years of irrigation on the area but hey HE is still alive, he is healthy, he continues with his profession, he is a GYN doctor and on top he lives in Cuba.

His surgeon was the best Dr. Shah, Jatin Head and Neck Oncology at the New York Memorial Sloan Kettering. I called him my family angel. HE is the best!!

There is hope, don't give up. It is better to be aggressive since the begining, don't try to go the easy way go aggressive like my dad did. He also didn't had a choice because the tumor was too advance he was terminal doctor gave him only a month to live and look 24 years later he is still here!! So please if you have any question about it ask me I am here to help!

I was treated for ENB in my sphenoid sinus in 2009-2010 at age 63.  I had multiple sinus surgeries, 33 radiation treatments with chemo, and four months of chemo.  I went through many of the pains and troubles described in this blog.  While grateful to be alive, radiotherapy left me with permanent collateral damage in several ways.

My worst problem is a complete inability to sleep naturally.  I never doze, nap, yawn, feel sleepy or sleep naturally.  Under the care of several fine doctors, I use various sleep aids nightly.  Nevertheless, I live with great fatigue.  I am otherwise in good health and have no anxiety issues.

I'd like to blog with any ENB survivors that have an extreme sleep issue. 

raym372 said:

Newly diagnosed with ENB
I have been diagnosed with Esthesioneuroblastoma in April. I went in for a nasal polyps removal, but when trying to remove, it was determined to be a tumor. They could only do a biopsy. Biopsy results showed Esthesioneuroblastoma. MRI showed my tumor size as 4.6x4.2x1.7. It is in my right nasal cavity and ethmoid sinus, and starting to extend into dura. They did not have enough tissue to determine my grade. My surgery is scheduled for next week at MD Anderson.

My doctors have determined that surgery followed by radiation of 6 weeks, as the current treatment plan. I will know more once they do the surgery and the grading.

My doctors will be doing the surgery all endoscopically. I have been told that they may do craniotomy if they do not get good margins endoscopically.

I have also read about elective neck irradiation (ENI), that might help in reducing recurrences. For those of you that had ENI, did that help in preventing recurrences, and did you have any side effects that persisted?

And did any of you also have chemo - either concurrently or after radiation?

Any suggestions and/or recommendations for the treatment and what I should be watching out for?

Ray

I had chemo followed by radiation before final surgery. I never did the ENI thing. All this was from JAN to JUL 2009. The docs told me up front that the chemo and radiation would not eliminate the tumor but only force it to encapsulate itself, making surgical removal easier and with much less blood loss. Chemo will make you sick, but they gave me antinausuals to help. You will also probably lose your hair (mine eventually grew back just fine).  The effects of radiation treatments will be cumulative. You'll feel fine for the frist 2 weeks but then get progressively weaker. I had to have someone drive me to treatment the last two weeks.  Since surgery in JUL 2009 I go back for a checkups annually. I'm coming up on 10 years.

raym372 said:

Newly diagnosed with ENB
I have been diagnosed with Esthesioneuroblastoma in April. I went in for a nasal polyps removal, but when trying to remove, it was determined to be a tumor. They could only do a biopsy. Biopsy results showed Esthesioneuroblastoma. MRI showed my tumor size as 4.6x4.2x1.7. It is in my right nasal cavity and ethmoid sinus, and starting to extend into dura. They did not have enough tissue to determine my grade. My surgery is scheduled for next week at MD Anderson.

My doctors have determined that surgery followed by radiation of 6 weeks, as the current treatment plan. I will know more once they do the surgery and the grading.

My doctors will be doing the surgery all endoscopically. I have been told that they may do craniotomy if they do not get good margins endoscopically.

I have also read about elective neck irradiation (ENI), that might help in reducing recurrences. For those of you that had ENI, did that help in preventing recurrences, and did you have any side effects that persisted?

And did any of you also have chemo - either concurrently or after radiation?

Any suggestions and/or recommendations for the treatment and what I should be watching out for?

Ray

I only had chemo and radiation treatment and have been cancer free since August 2003.  The chemo very nearly completely eliminated the tumor even though it was in very late stages of development.  I did not have surgery as the followup radiation treatment destroyed what was left of the tumor.  The chemo made me a little nauseous, but was not debilitating, and the radiation didn't really affect me at all other than drying out my sinuses.  I did lose my hair, but it grew back just fine.  I have had no major issues since then.  The only long term effects have been dry sinuses and permanent anosmia, which oddly, has not affected my sense of taste.

Just wanted to provide an update for those who are newly diagnosed.  I am coming up on 5 years after my surgery to remove my esthesioneuroblastoma.  I lost my sense of smell after the surgery but no other effects due to surgery.  The radiation to my face caused my tears to not drain properly but I have since had a surgery to correct that as well.  My most recent MRI shows no sighs of the tumor returning.  Hopefully, it will stay that way.  Feel free to reach out.  I don't mind sharing my experience if you want to talk to someone who has been through it.  

Mona4 said:

Update- Sept 2019 5 year survivor

Just wanted to provide an update for those who are newly diagnosed.  I am coming up on 5 years after my surgery to remove my esthesioneuroblastoma.  I lost my sense of smell after the surgery but no other effects due to surgery.  The radiation to my face caused my tears to not drain properly but I have since had a surgery to correct that as well.  My most recent MRI shows no sighs of the tumor returning.  Hopefully, it will stay that way.  Feel free to reach out.  I don't mind sharing my experience if you want to talk to someone who has been through it.  

God bless, Mona.  I am so happy for your continued good health.  It has been almost 17 years for me, and so far so good.  Unfortunately, if you are like me, the sense of smell will likely never return.  However, that is not always a bad thing!   Love to you and may you have peace in your life.